Pancreatic cancer screening should be considered in patients with genetic syndromes associated with an increased risk, including Peutz-Jeghers syndrome, hereditary pancreatitis, Cyclin Dependent Kinase Inhibitor 2A (CDKN2A) gene mutation, Lynch syndrome, and mutations in breast cancer 1 (BRCA1), BRCA2, partner and localizer of BRCA2 (PALB2), and ataxia-telangiectasia mutated (ATM) genes. Here, BRCA1 is linked to Peutz-Jeghers syndrome.