Patients with HNF1B-MODY may present renal cysts in 62–83% of cases, pancreatic atrophy, diabetes in 48% of cases, urogenital malformations such as Mayer–Rokitansky–Küster syndrome and hypospadias, hypomagnesemia in 48% of cases, hyperuricemia, and hyperparathyroidism [12, 14] Approximately 50% of cases can be associated with deletions in this gene [15]. The gene discussed is HNF1B; the disease is cystic kidney disease.