Several genetic syndromes, such as Zellweger syndrome, VACTERL association, Eagle-Barrett syndrome, renal–hepatic–pancreatic dysplasia (RHPD), Branchio-oto-renal (BOR) syndrome, HNF1B nephropathy and renal coloboma syndrome (RCS), have been linked to kidney cystic disease [9, 18, 23, 26, 27]. This evidence concerns the gene HNF1B and kidney disorder.