The management of 27 patients who presented with cholangiocarcinoma of the hepatic confluence over the past 20 years is reviewed, and the dramatic changes, both in accuracy of diagnosis and modes of treatment, are documented. All 27 presented with jaundice, three underwent laparotomy only, four had a T-tube inserted, one had a straight transhepatic tube inserted, seven U-tubes were placed and four patients underwent surgical resection. More recently, seven patients were drained percutaneously and a further patient was treated by an endoscopic stent. There were six postoperative deaths. Of the 24 who received active treatment, the mean survival was 8 months with a range of 0-40 months. The advantages and disadvantages of the various methods of diagnosis and treatment are discussed. The possible role of radiotherapy and chemotherapy are reviewed briefly.