35 answers

Toddler's Pee Smells like Maple Syrup

My husband and I have been trying to figure out why our 20 month old daughter's clothes sometimes smell like maple syrup - this weekend, she took off her clothes during her naptime and peed all over them - they all smelled like maple syrup. Has anyone ever heard of this? She has a very healthy, varied diet and has never been sick except for one cold last winter.

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Thank you everyone for the great response!! I spoke to our ped. He asked a lot of questions and will test her, but first he wants to see her infant screening results. He never received them because the hospital would not send the results to him until the adoption was complete - the adoption was finalized a year ago, but we didn't think about it till now. The hospital is in San Diego and we're in Glendale, so I faxed the order yesterday. Meantime, we're feeding her less protein and more carbs because that's what all the websites seem to indicate. She is as lively and uncannily clever as she always is, but we're watching her like hawks! I'll update you again after we do the blood work. Thank you all again. Mamasource really is a wonderful community!

Featured Answers

Do you take fenugreek (a supplement to help with milk supply)? I know it always made me (not my pee, that I remember) smell like maple syrup!

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are you breastfeeding her? If so, are you taking "fenugreek" to increase milk supply? Because that is known to make you and/or baby to smell like maple syrup!

1 mom found this helpful

I'm not 100& perecent about this but i think i remember someone mentioning this is a reaction to diabetes before.

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More Answers

Do you take fenugreek (a supplement to help with milk supply)? I know it always made me (not my pee, that I remember) smell like maple syrup!

2 moms found this helpful

I'm not 100& perecent about this but i think i remember someone mentioning this is a reaction to diabetes before.

1 mom found this helpful

Hi L.,
Take her to the doctor to have her evaluated for a metabolic dsorder that is sometimes called, "maple syrup urine disease." It's genetic and treatable with diet, but can be serious. Good for you for figuring it out! E.

1 mom found this helpful

While it could just be a function of something in her diet, generally speaking, urine that smells sweet like maple syrup is a classic sign of diabetes. In those cases, the body isn't producing insulin to metabolize sugar, so the sugar in the body is eliminated through urination. Obviously there are other effects of diabetes, but I would speak to your doctor about it. Before you worry too much just remember that the smell and color of urine is easily effected by diet, so the doctor may say it's nothing. But better safe than sorry! Good luck!

1 mom found this helpful

Do you use the herb fenugreek in your cooking or is it in any supplement she is taking? I took Fenugreek pills to help lactation while I was nursing my daughters and it will make your pee, underarm, sweat etc. smell like pancake syrup. I hope this info helps!!

1 mom found this helpful

did you find out the cause? my daughter has the same problem.

1 mom found this helpful

are you breastfeeding her? If so, are you taking "fenugreek" to increase milk supply? Because that is known to make you and/or baby to smell like maple syrup!

1 mom found this helpful

L., I'm not sure that I agree with the other moms that have posted. Your baby seems a little old to have maple syrup urine disease. I have twin boys that just turned 22 months and I have noticed with both of them that their urine if left on their clothes will start to smell sweet like maple syrup and they are both healthy as can be. They both do eat lots of protein and large amounts of fruit...I'm sure its all about the diet.

Hi L.,
There is something called Maple Syrup disease (I think that's what it's called). It is a metabolic disorder although I wouldn't panic.....my son used to have that same smell on the back of his neck when he would sweat. I mentioned it to the doctor and they acted as if we would know (he would have some other symptoms)if he had this. Definitely look into it since her urine smells like maple syrup but as I said don't panic.

my sons clothes smells like that sometimes.. even when he does not pee.. mostly when he wakes up in the morning. He is almost 4 years old and whenever they do tests they don't tell me anything so I am just assuming its sweat. Let me know if you find out more.

Ask the DR to do a urine test. Sugar in the urine can be diabetes -- don't wait, test to be sure.

Not to alarm you but if you want more information about MSUD google "the clinic for special children" it is in Lancaster PA and they specialize in this rare disorder.

Good question- My daughters does too. Or at least her clothes do when they get damp from a full diaper, especially over night. I am looking forward to someone who knows the answer to why and what causes this.

My husband was taking a biology class and not too long ago and was telling me about something called "Maple Syrup Urine Disease". I thought he was kidding at first but you should check into it. Apparently it's some type of chromosome abnormality and can be serious if not treated. Good Luck.

L., I do think you should see her dr. but also wanted to let you know that I have also heard that sometimes it can be a caused by what they are eating. Ketones have this sweet smell, it can happen if they eat a lot of fruit, low-no carbs, high protein or if they get a little dehydrated. There are other conditions this can be caused by but I think most of the time it's what above. Good luck and let us know what you find out.

Go to the hospital immediately, if not sooner. now now now. that is so totally bad that you need to go NOW!

Have your doctor screen for maple syrup urine disease.
Best of luck!

Thats normal, my kids smelled the same and Dr. said nothing to worry about. I think it is the pee mixed with sweat that causes that smell. Pull ups are your best bet to save on all the washing. Good luck!

Please call your doctor and tell him about this. There is a genetic disease that is called Maple Syrup Urine Disease that can be fatal if not diagnosed early (within the first mopnths of life). You can look it up on the web. There is treatment but needs to be diagnosed early. I don't want to scare you but PLEASE call your doctor and let him know. Time is of the essence.



This site will give you a little information on the Maple syrup Disease. Tell your physician. It has to do with genes and it a rare disease. If she has it she needs to go on a special diet. There are mild and severe cases but it proteins build up in the blood stream and cause brain damage. Be safe and have her tested. It will also give you peace of mind to know if she does or does not have it.

Oh my gosh....going through this right now with my 14 mos old son. We brought it up at his 12 mos appt and he did a lactic and pyruvic acid test. Which were off (just to mention his newborn screening came back normal). Then we did uring organic amino acids and they were off as well. Then we did a CPK and PH level which were off. They are telling me he has mild persistant acidosis. Whatever that means! He is going to have a bunch more tests tomorrow and then possibly going to a children's hospital to see a specialist. SO make sure you ask for lactic, pyruvic amino acids, possibly urine organic amino acids, a CPK and ph level. Good luck

You should check with your pediatrician. Not sure if it was medically related, but my best friend growing up always smelled like maple syrup. If you smelled any of her bedding, clothes, etc., always smelled like maple syrup. We all just assumed it was her natural scent.

Don't want to scare you but you should look up "Maple Syrup Urine Disease." From what I've read it's treatable but could have neurological implications if left untreated. You should have her checked out if you think the symptoms fit - it may not be anything but with something like this it seems like a definite reason to visit your doctor.
Good luck!

I've heard that this might indicate problems with sugar metabolism. Please check with your doctor.

Contact your physician NOW.
There is a rare metabolic disorder (that can be treated) called Maple Syrup urine disorder.
Best wishes for you and yours...And bravo for asking...most moms would dismiss it as just an odd smell in the house.

You need to inform your pediatrician. It is probably nothing, but there is something called MSUD. http://www.nlm.nih.gov/medlineplus/ency/article/000373.htm

Hi L.,

In order to be on a safe side, please take your daughter to her pediatrician and ask to run a Blood Sugar test. The best way is probably to run a urine test. Good Luck!

My son has smelled like this before too. I remembered learning about some crazy disorder that cab cause this, so naturally I was really worried. I took him to the dr. and he was fine. So try not to alarm yourself, it could be-- probably totally is NOTHING.

Does she seem thirsty all the time? Is she urinating frequently? You might want to get her tested for type 1 diabetes. Our daughter was dxd at 23 months. Undiagnosed diabetics have sugar in their urine, which may be causing the syrupy smell? All it takes is a blood test. Like your daughter, our daughter had never been sick before, either.

I would call your doctor. There is a condition called MSUD (Maple Syrup Urine Disease). In a previous job, one of my clients had this. It's a rare metabolic disease. Good luck!

It can be a rare metobolic problem. You should have your baby checked out immediately.

talk to your pediatrician. It may just be something she ate and how she processes it (my daughter had similiar episodes).. however there are also specific medical conditions that could cause this sweet smelling urine (don't get nervous, just get a some professional assesment to make sure you're not overlooking something).

Probably nothing, but check this out..........good luck.Maple syrup urine disease
Read More
Amino acids
Seizures Maple syrup urine disease (MSUD) is a metabolism disorder passed down through families in which the body cannot break down certain parts of proteins. Urine in persons with this condition can smell like maple syrup.

Maple syrup urine disease (MSUD) is caused by a gene defect. Persons with this condition cannot break down the branched-chain amino acids leucine, isoleucine, and valine. This leads to a buildup of these chemicals in the blood.

In the most severe form, MSUD can damage the brain during times of physical stress (such as infection, fever, or not eating for a long time).

MSUD also occurs in an intermittent form and a mild form. Even in the mildest form, repeated periods of physical stress can cause mental retardation and high levels of leucine.

Avoiding food
Feeding difficulties
Urine that smells like maple syrup

Definitely you need to get her tested for diabetes! Good luck.

Several people have already mentioned the herbal supplement "fenugreek." Are you taking it?

When I took that to increase my milk supply, my pee smelled like maple syrup. (Seriously TMI, lol.)

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