Mutations in the high penetrance genes such as BRCA2, PALB2, p16/CDKN2A, PRSS1, SPINK1, and STK11 correlate with a very high lifetime risk of developing pancreatic cancer and may cause as many as 10% of pancreatic cancers in the US [19]. The gene discussed is PRSS1; the disease is familial pancreatic carcinoma.