Hilar cholangiocarcinoma is relatively rare. Typically it spreads locally, the main manifestation being painless jaundice. Exact preoperative staging includes sonography or CT scan, cholangiography, angiography and aspiration cytology. This assessment should delineate the tumor extent and provide an excellent index of tumors which are clearly unresectable. Therapy has two objectives: firstly, if possible, to eliminate the tumor, and secondly, to restore bile flow. Resection of hilar cholangiocarcinoma can be accomplished in some 20% of cases with a 30 day hospital mortality close to zero for local excision and of approximately 10% for hepatic resections. The median time resected patients remain alive is 24 months, and the 5 year survival rate of resected patients is 12%. The mortality rate for palliative relief of biliary obstruction by any method in hilar cholangiocarcinoma is reported at 16-30%. Survival after intubation averages only 3 to 6 months and for surgical bypass about 8 months.