In BRCA1/2 mutations, there are co-mutations in high frequency with other genes that are involved in DNA repair, which include KRAS, MET, and MDM2/6 [9]. However, to our knowledge, this is the first reported case of cholangiocarcinoma with mutations in both BRCA1 and BRCA2. This evidence concerns the gene BRCA1 and cholangiocarcinoma.