We present a 56-year-old male patient affected with metastatic ICC...A novel BAP1 mutation (splice site c.581-17_585del22) and a RAD21 amplification were identified...patient was treated with PARP inhibitor olaparib. The patient had a partial response....with an overall survival of 37.2 months from the time of diagnosis of his ICC. Following 11.0 months on olaparib treatment, sustained stable disease control is ongoing.