Abstract: Background Hilar cholangiocarcinoma (HC) originates in the main extrahepatic left or right duct or their confluence It’s still considered to be hard to treat or cure.Data source:We reviewed the medical literatures on HC and we analysis the relevant and updated information in a concise and easy-to-read manner.Result The selection and timing of management options for HC are determined by the diagnosis,the underlying liver function,the general condition of the patients and the stage of the disease.Nowadays,the treatment of HC can be divided into curative and palliative treatment.For the curative treatment,local excision should only be used on Bismuth I papillary carcinoma.Combined partial hepatectomy should be combined with caudate lobe resection and porta-hepatis lymph node dissection.The results of these surgical selections can be improved by portal vein embolization,staging laparoscopy and laparoscopic ultrasound.The role of preoperative biliary drainage is controversial.Autotransplantation for HC gave disappointing results while the Mayo Protocol of chemoradiation for selecting patients with unresectable HC for orthotopic liver transplantation has been widely accepted.Palliative treatment included bypass surgery,endoscopic or percutaneous stenting,photodynamic therapy,intraluminal brachytherapy,and external radiation and systemic therapy.Conclusions:Adequate surgery with R0 resection should be the main goal of treatment.For patients with unresectable HC,treatment aims to improve the quality and quantity of their survival.

Key words: Hilar cholangiocarcinoma, Curative therapy