Maturity on-set diabetes of the young is a form of diabetes characterized by a mutation in a single gene. Whereas Type 1 diabetes is an autoimmune disorder which attacks insulin producing cells and Type 2 diabetes is polygenic and is influenced by environmental and lifestyle factors.

MODY is very rare and usually presents in individuals under 30 years old with strong family history. There are numerous types that have been identified and genetic testing is required for diagnosis.

MODY4 represents about 1% of all reported MODY cases so very little clinical information is available.