Because the prevalence of ADPKD in the general population is 1 in 500–1,000 (Torres et al., 2007) (whereas PKD2 genetic prevalence is estimated at 2.31/10,000) (Choi et al., 2014) and the prevalence of NF1 is approximately 1 in 3,000 (Ward & Gutmann, 2005), concurrence of both diseases (ADPKD and NF1) is predicted to occur in 1 in 1,500,000–3,000,000 individuals; concurrence of PKD2 disease and NF1 disease is predicted to occur in 1/13,043,400. Here, PKD2 is linked to polycystic kidney disease 2.