Hilar cholangiocarcinoma still has a poor prognosis and a relatively low 5-year survival rate despite improvement of the resection rate and curative resection rate. Optimal surgical strategies should be chosen according to the balance between operation safety and curative resection. Extended resection and dissection of lymph nodes do not always ensure successful treatment. Preoperative evaluation of liver function should be carefully carried out. For patients with severe jaundice or those who undergo extensive hepatic resection, preoperative biliary drainage, portal vein embolization and a precise hepatec-tomy technique should be carried out to improve the safety of the operation. Assessment of resectability should be carried out to avoid unnecessary laparotomy, and endoscopic drainage is preferable in dealing with unresectable tumors. The aim of palliative treatment is to relieve biliary obstruction, prolong life and improve the quality of life. Biological characteristics of hilar cholangiocarcinoma need to be taken into account and multi-modality therapy is required to improve treatment. The survival rate of patients with hilar cholangiocarcinoma may be improved by comprehensive treatment. Populations with high risks should be monitored and screened to find patients at early stages of the disease.