Hilar cholangiocarcinoma is the most common extrahepatic malignant tumor in clinic, accounting for about 50%-60% of bile duct tumors. Currently, radical surgical resection is regarded as the best treatment for early hepatic hilar cholangiocarcinoma, but, it’s early lesions have no obvious clinical symptoms. Most patients are already in the advanced stage of the disease when they are admitted to hospital. The surgical resection rate is low, and the 5-year survival rate does not exceed 25%. Locally advanced hepatic hilar cholangiocarcinoma are treated with neoadjuvant chemoradiation therapy followed by surgery, it can prolong survival time of the patient. At the same time, the recurrence rate after surgery can reach 50%-70%, and the cancer easily invades microvessels, lymphatic vessels, peripheral nerves and liver, which is one of the most challenging problems in the field of biliary surgery. Therefore, early diagnosis, perioperative management, adequate and accurate preoperative staging assessment, intraoperative standardized resection, postoperative adjuvant therapy of hilar cholangiocarcinoma, it is of great significance to improve the rate of radical surgical resection and prolong the survival time of patients.