Herein, we report a case of an ADPKD family, in which the youngest male member was referred to the department of nephrology in the age of twenty with an uremic syndrome and ESRD due to ADPKD, and in which bilineal inheritance of pathogenic PKD1 and PKD2 variants has been revealed based on genetic studies. Here, PKD1 is linked to autosomal dominant polycystic kidney disease.