Here, we present a case of metastatic pancreatic adenocarcinoma harboring a germline BRCA1 mutation and a high tumor mutation burden (TMB), demonstrating an excellent response to initial platinum-based chemotherapy, followed by a complete radiologic response to maintenance immunotherapy and poly (ADP-ribose) polymerase (PARP) inhibition. The gene discussed is BRCA1; the disease is neoplasm.