Objective To summarize the progress in diagnosis and treatment of hilar cholangiocarcinoma at present. Methods The literatures about diagnosis and treatment of hilar cholangiocarcinoma at home and aboard were collected to make an review. Results The diagnosis of hilar cholangiocarcinoma mainly depended on serum tumor molecular markers and imaging examinations. Preoperative excision and prognostic evaluation were required, including tumor classification and staging, preoperative yellow reduction, residual liver volume assessment, and so on. Radical resection was the first choice, as well as liver transplantation, radiotherapy, chemotherapy, and photodynamic therapy could be selected according to the patient’s condition. Conclusions The appearance of new diagnosis and treatment technology promotes the clinical development of hilar cholangiocarcinoma. The integrated diagnosis and treatment mode, which is based on surgery, will become the inevitable direction of the development of hilar cholangiocarcinoma.