An estimated 10% of pancreatic cancer cases are associated with inherited predisposition, based on familial clustering.13 Several germline mutations have been linked to familial pancreatic cancer.14 The role of germline mutations in several genes, such as INK4A, BRCA2, and LKB1, and their associations with pancreatic cancer risk is beyond the scope of this review. This evidence concerns the gene STK11 and familial pancreatic carcinoma.