The term ‘Maturity-Onset Diabetes of the young’ (MODY) was first described in 1976 and is currently referred to as monogenic diabetes. There are 10 known entities accounting for 1–2% of diabetes and are frequently misdiagnosed as either type 1 or 2 diabetes. Correct diagnosis of monogenic diabetes has implications on managing patients and their families. MODY-5 is an entity that is associated with genitourinary malformations and should be considered by obstetricians in pregnant women with a family history of diabetes, genitourinary malformations fetal renal anomalies and a screen positive for diabetes. We are reporting a case of a 21-year-old pregnant patient with a bicornuate uterus, high HbA1c at antenatal screening and fetal renal anomalies that were suggestive of a MODY 5 diabetes.