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An estimated 10% of individuals with pancreatic cancer have an inherited predisposition to the disease. Germline mutations in BRCA2, p16, STK11, and the cationic trypsinogen gene contribute to inherited forms of pancreatic cancer, but the gene(s) responsible for much of the familial pancreatic cancer burden remains to be identified. The high lifetime risk of pancreatic cancer that exists among at-risk members of families with multiple pancreatic cancers highlights the pressing need for pancreatic cancer early detection strategies. Since curative pancreatic resection is still the only curative treatment for most patients with pancreatic cancer, the early detection of symptomless pancreatic cancer using endoscopic ultrasound or molecular markers may provide the best chance of cure for individuals at high risk of this disease.