Tier I or II mutations related to hereditary cancer syndrome in pancreatic cancer were identified in BRCA1 (n = 2, 2.3%), BRCA2 (n = 2, 2.3%), PRSS1 (n = 1, 1.1%), MSH6 (n = 2, 2.3%), PMS2 (n = 1, 1.1%), and APC (n = 1, 1.1%). The gene discussed is MSH6; the disease is familial pancreatic carcinoma.