A 57-year-old woman who was diagnosed with cholangiolocellular carcinoma underwent neoadjuvant chemotherapy with gemcitabine (GEM). The clinical stage was cT3N1M1 (right adrenal grand),cStage IVB (JPS) with invasion to the inferior vena cava (IVC). We were willing to perform hepatectomy if the response to chemotherapy was stable disease (SD) or better according to the Response Evaluation Criteria In Solid Tumors. After 2 courses of preoperative chemotherapy with GEM, SD was obtained. She underwent right lobectomy of the caudate lobe and resection of the right adrenal gland. The IVC was also resected and reconstructed. The pathological diagnosis was as follows: cholangiolocellular carcinoma, im (-), eg, fc(-), sf(-), s0, n0, vp0, vv0, va0, b1, p0, sm(-), 200 μm, pT2N0M0, pStage II, and Cur A2. The IVC was not invaded and the right adrenal gland was diagnosed with adrenocortical adenoma. She underwent continuing adjuvant chemotherapy with GEM for 4 months without recurrence. Therapy for cholangiolocellular carcinoma has not yet been established, but multimodal treatment with GEM and surgical resection is potentially effective for cholangiolocellular carcinoma.