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]. This evidence concerns the gene HNF1B and autosomal dominant polycystic kidney disease.