Autosomal recessive polycystic kidney disease (ARPKD) is a rare and severe renal cystic disease caused by homozygous or compound heterozygous variants of polycystic kidney and hepatic disease 1 (PKHD1) or DAZ interacting zinc finger protein 1 like (DZIP1L) (1, 2). This evidence concerns the gene DZIP1L and urogenital neoplasm.