OBJECTIVE To explore the feasibility and outcome of combined hepatectomy, orthotopic liver transplantation and Whipple's pancreatoduodenectomy for radical excision of cholangiocarcinoma. METHODS A 35-year-old female patient with unresectable cholangiocarcinoma underwent curative radical operation, which includes hepatectomy, orthotopic liver transplantation and pancreaticoduodenectomy of hilar bile duct carcinoma; immunosuppression followed an induction protocol with FK506 and steroids. Lamivudine and HBIg were used to prevent hepatitis B virus from infection again. RESULTS Pathologic examination revealed low differentiated cholangiocarcinoma and immunohistochemistry stains demonstrated positive expression of cytokeratin 9 and 17, carcinoembryonic antigen and the hepatocyte protein was negative. Neither the margins of resection nor the periductal lymph nodes were involved. The liver showed evidence of cholestasis and metastasis nod. The patient was hospitalized 32 days and came back to the previous work. He has been followed up for more than 14 months up to now and is currently alive without any evidence of recurrent cancer. CONCLUSIONS For some selected unresectable Klatskin's tumors, combined hepatectomy, pancreatoduodenectomy and orthotopic liver transplantation was justified. The radical methods maybe provide long-time survival and curative effect. Nevertheless, because of possible tumor recurrence and ethical controversy, the combined hepatectomy, pancreatoduodenectomy and orthotopic liver transplantation procedure has to be applied only with caution and indications.