Autosomal dominant polycystic kidney disease (ADPKD) occurs with an incidence of ~1:1000 in all ethnic groups and develops as the result of mutations in the PKD1 (~70–85%) or PKD2 (~15–30%) gene, which encodes the protein product polycystin-1 (PC1) or polycystin-2 (PC2), respectively1. The gene discussed is PKD2; the disease is autosomal dominant polycystic kidney disease.