The seven probands with inherited renal structural abnormality included four autosomal recessive polycystic kidney disease (ARPKD) caused by the compound heterozygous variants in PKHD1 and three autosomal dominant polycystic kidney disease (ADPKD) caused by the heterozygous variants in PKD1. This evidence concerns the gene PKHD1 and autosomal dominant polycystic kidney disease.