Genetic mutations of damaged DNA repair genes such as BRCA1/2, partner and localizer of BRCA2 (PALB2), and the ataxia-telangiectasia mutated (ATM) gene along with the cyclin-dependent kinase inhibitor 2A (CDKN2A) gene (familial atypical multiple mole and melanoma syndrome) and mismatch repair genes (Lynch syndrome) are associated with significantly increased risk of pancreatic cancer. Here, CDKN2A is linked to familial pancreatic carcinoma.