Only mild anemia and immune-related thyroiditis were observed, which were easily manageable and did not result in discontinuation of olaparib and pembrolizumab.<h4>Conclusion</h4>The presented case showed substantial clinical activity of a combination with olaparib/pembrolizumab in advanced BRCA2-mutated CCA. The gene discussed is BRCA2; the disease is cholangiocarcinoma.