We report the case of a 52-year-old woman with cholangiocellular carcinoma (CCC) involving the lymph node and lung metastases who was admitted to our hospital because of right abdominal pain and anorexia. We started systemic chemotherapy(SCT) comprising 5-fluorouracil(5-FU; 1 g) and gemcitabine(GEM; 1 g) biweekly. After 1 course of SCT, we treated her with high-dose hepatic arterial injection (6 g 5-FU/1 week). This chemotherapy regimen improved her symptoms. After placing an arterial port into the right femoral artery, we continued SCT comprising GEM biweekly and hepatic arterial injection(HAI) of 5-FU weekly. Finally, we opted for weekly HAI and simultaneous SCT of 5-FU and GEM biweekly. The SCT dose of 5-FU was 1,000 mg and the dose of GEM ranged from 800 to 1,000 mg. For HAI, the dose of 5-FU was changed to between 250 and 750 mg. Lymph node metastases disappeared and lung metastases were reduced by this treatment. Because the lung metastases had progressed after 10 months, we increased the SCT dose of 5-FU to 1,500 mg and the dose of GEM to 1,200 mg. We also added systemic mitomycin C and prolonged the injection time of systemic 5-FU from 3 hours to 3 days. The lung metastases were reduced by this treatment. Our patient is still alive and has worked for more than 1 year. We recommend combination therapy of SCT and HAI for CCC with metastases.