In addition to VEO ADPKD, the broad differential diagnoses include, e.g., Bardet-Biedl syndrome (BBS), HNF1B-nephropathy, cystic kidney dysplasia, infantile nephronophthisis, and metabolic disorders including defects of fatty acid oxidation [2, 7, 10]. Here, HNF1B is linked to Bardet-Biedl syndrome.