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. The gene discussed is PKD1; the disease is autosomal dominant polycystic kidney disease.