Previous studies showed that severe prenatal PKD patients may carry, in addition to the inherited PKD mutation, a co-inheritance of ADPKD and ARPKD (oligogenic model of PKD) or other cystogenic genes such as HNF1B (15) or a ciliopathic gene such as IFT140 (31). Here, HNF1B is linked to autosomal recessive polycystic kidney disease.