【Abstract】ObjectiveTo report the diagnosis and treatment of hilar cholangiocarcinoma.MethodsThe relevant information about the hispathological feature, transfer ways, clinical manifestation, laboratory examination, imaging feature, immunohistochemical examination and treatment ways were gathered from previous original articles, and checking the latest issues of appropriate journals.ResultsThe clinical manifestation, laboratory examination, and imaging feature of hilar cholangiocarcinoma were due to the neoplasm obstructing bile duct and sequent infection of bile duct. The diagnosis was depanded on the combining clinical manifestation, laboratory examination and imaging feature. The value of immunohistochemical examination was not clear. Radical surgery was the best treatment of unique curing the neoplasm. By-pass surgery was used in the late phase patients to solve the obstruction of bile and digest duct. The effect of unique chemical treatment was not perfect. It did’t generally propose the treatment of orthotopic liver transplantation.ConclusionThe perfect prognosis of hilar cholangiocarcinoma is depended on early diagnosis and redical surgery.