ADPLD affects 1/100,000 individuals worldwide, while ADPKD has a prevalence of 1/1000 people; a rare form of autosomal recessive polycystic kidney disease (ARPKD) affects 1/10,000 to 1/40,000 individuals [4] and is associated with congenital hepatic fibrosis rather than liver cysts (PKHD1, DZIP1L, CYS1, PKD1) [5]. This evidence concerns the gene PKHD1 and autosomal recessive polycystic kidney disease.