The main representatives of this group of diseases are autosomal recessive polycystic kidney disease (ARPKD), nephronophthisis and nephronophthisis-related ciliopathies (NPH/NPH-RC), Bardet–Biedl syndrome (BBS), and hepatocyte nuclear factor-1beta (HNF1B) nephropathy (1). This evidence concerns the gene HNF1B and Nephropathy.