The differential clinical and genetical diagnosis in children with ARPKD suspicion must include diseases that can imitate the ARPKD phenotype, like very early onset of autosomal dominant polycystic kidney disease (VEO-ADPKD), hepatocyte nuclear factor 1β (HNF1B) nephropathy, or nephronophthisis (NPHP). Here, HNF1B is linked to Nephropathy.