The deleted segment encompassed 16 known genes, including TCF2 and LHX1. Mutations in TCF2 gene, also known as hepatocyte nuclear factor-1-beta (HNF1β), which occur in individuals affected by maturity-onset diabetes of the young type 5 (MODY5; MIM 137920) and renal manifestations [15], have been associated with Müllerian disorders [1]. The gene discussed is LHX1; the disease is renal cysts and diabetes syndrome.