We presented a case of a patient with advanced intrahepatic cholangiocarcinoma (iCCA) harboring dual somatic homologous recombination repair (HRR) gene pathogenic variants, specifically BRCA1 and PALB2, who achieved PR lasting approximately 7 months following salvage treatment with olaparib. Here, BRCA1 is linked to infantile convulsions and choreoathetosis.