Autosomal dominant polycystic kidney disease (ADPKD) is an inherited disease caused by mutations in two genes, PKD1 and PKD2, which encode for polycystin 1 and polycystin 2, respectively: mutations in PKD1 are present in 85% of cases, and mutations in PKD2 in the remaining 15% [1-3]. Here, PKD2 is linked to autosomal dominant polycystic kidney disease.