Genetic Disorders that Cause Stinky Urine
Maple Syrup Urine Disease

Maple Syrup Urine Disease, or branched-chain ketoaciduria, is a rare genetic disease that causes problems with metabolizing branched chain amino acids. It causes a baby's urine to smell like maple syrup or burnt sugar.
Phenylketonuria
Phenylketonuria, also known as PKU, is a rare genetic disorder that may cause smelly urine. The smell is often described as "mousy" or "musty" and is usually detected not only in urine, but in body odor and breath as well.
Glycogen Storage Disease
Glycogen storage disease type 1, or GSDI or von Gierke disease, is a genetic disorder. That means that if your parents or grandparents had it, you may be at risk.
​
It starts early, when you’re still an infant, and it can lead to problems with growth later in life. If left unchecked and untreated, it could lead to problems with glycogen being stored as fat, which can prove fatal.
Renal Glycosuria
Renal glycosuria is a very rare disease when it’s inherited, and it’s estimated to only affect 0.16-6.3% of the total population.
​
The condition is benign, which means that people who have it don’t have many problems, and cases of hypoglycemia or hypoyolemia don’t occur often.