Worst Case Scenarios: Is Stinky Urine a Sign of a Disease?
Maple Syrup Urine Disease



Phenylketonuria

Glycogen Storage Disease

Bladder Cancer
If you are having problems with smelly urine, you may be worried about whether it is a sign of a disease or health problem. This is especially true if you are also having other symptoms you believe might be related to the bad smell in your urine.
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The only way to find out for sure what is causing the bad odor in your urine is to consult your doctor. However, knowing the worst possibilities up front can help you be prepared and informed so you can make the most of your doctor visit.
These causes are much less likely than the possibilities on our Top 10 Most Common Causes of Stinky Urine list, but they are possibilities nonetheless.

Fistula

Liver Failure

Ketoacidosis

Diabetes