Interestingly, ADPKD in this family was originated by a truncating mutation in PKD2 and is a fact that the proband`s mother, proband`s aunt and proband`s grandmother presented ESRD at age of 49, 60, and 70, respectively, which may be considered as intra‐familial renal disease variability for PKD2 and may suggest the presence of a modifier effect for rapid progression. This evidence concerns the gene PKD2 and autosomal dominant polycystic kidney disease.