Histological examination of paraffin-embedded (FFPE) specimens from surgically removed tumor tissue led to a pathological diagnosis of stage II (cT2N0M0) ICC...The patient was identified to harbor four somatic genetic variations, including BAP1 mutation (Q40*), IDH2 mutation (R172W) as well as the co-deletion of CDKN2A and CDKN2B genes...The palbociclib treatment (125 mg daily for 21 days, then 7 days off) was subsequently started...These indicated that the patient had a good response to palbociclib treatment.