High-risk individuals (HRIs) for pancreatic ductal adenocarcinoma (PDAC) include patients with a history of familial pancreatic cancer (FPDAC) or pathogenic germline mutations such as STK11, CDKN2A, BRCA1/2, ATM, PALB2, MLH1, MSH2, MSH6, EPCAM, and TP53. This evidence concerns the gene MLH1 and familial pancreatic carcinoma.