As a differential diagnosis ADPKD, congenital nephrosis of the finnish type, Meckel Gruber syndrome, Trisomy 13 and Beckwith Wiedemann, as well as loss of HNF1B can appear prenatally with a similar phenotype with enlarged hyperechogenic kidneys [30]. The gene discussed is HNF1B; the disease is autosomal dominant polycystic kidney disease.