The genetic etiology of cystogenesis in individuals with a clinical diagnosis of ADPKD may well range beyond variants in PKD1 and PKD2. ARPKD is more frequently considered in the evaluation of perinatal hepatic and kidney disease, given the many reports of early-presenting ARPKD in the literature (28, 29). Here, PKD1 is linked to autosomal recessive polycystic kidney disease.