The most common known forms of diabetes are type 1, an autoimmune disorder with auto antibodies and type II which is multi-factorial influenced by genetics, environment. Another form of diabetes is MODY (maturity onset diabetes in young), the most common form of monogenic diabetes, seen in non-obese children, adolescents and young adults. MODY a rare condition, accounts for 1–5% of all cases of DM and 1–6% of paediatric diabetes. This is non-ketotic and patients have no antibodies. Hepatocyte Nuclear Factor-1Beta is one of the several genes associated with this disease, causing beta cell dysfunction and extrapancreatic manifestations involving kidney, liver and intestines. In the present case glomerular cystic kidney disease is identified in a young male with diabetes raising clinical strong suspicion of maturity onset diabetes, which later showed Hepatocyte Nuclear Factor-1Beta mutation. A 17-year-old male, presumed type-1 diabetic who was under regular follow up with an endocrinologist, presented with elevated serum creatinine and protein in urine. On referral to our nephrologist, further evaluation was done when cortical kidney cysts were observed on imaging and on biopsy glomerular cystic kidney disease was identified. Genetic analysis revealed heterozygous missense variant of Hepatocyte Nuclear Factor-1Beta on Exon 4, thereby a final diagnosis of maturity onset diabetes in young-hepatocyte nuclear factor-1beta considered. His condition was stable during follow-up, despite the fact that chronic kidney disease is a progressive illness. Interdisciplinary approach helps in accurate typing of diabetes which has a bearing on prognosis and treatment.