We identified a hemizygous whole-gene deletion in the HNF1β gene in a 17-year-old girl with nephropathy, renal cysts in the left kidney, and absence of a right kidney as identified by computed tomography scans, along with loss of the vagina after following the clinical characteristics of high glucose levels and impaired insulin secretion. The gene discussed is HNF1B; the disease is Renal cyst.