Carriers of germline BRCA1/2 pathogenic variants are at increased risk of developing BRCA-associated malignancies (breast, ovarian, prostate and pancreatic cancer), which are responsive to treatment with poly (adenosine phosphate-ribose) polymerase inhibitors (PARPi) and platinum-based chemotherapy (PBC). The gene discussed is BRCA1; the disease is familial pancreatic carcinoma.