For patients with irresectable hilar cholangiocarcinoma, liver transplantation (LT) is currently being reassessed because of promising data for neoadjuvant radiochemotherapy. For increased radicality, hepatectomy in combination with pancreatic head resection [extended bile duct resection (EBDR)] was performed for irresectable hilar cholangiocarcinoma during our initial experience. EBDR and LT was performed in 16 patients between 1992 and 1998. No neoadjuvant or adjuvant treatment was performed. The Union Internationale Contre le Cancer stages were I (n = 6), IIA (5), IIB (3), and IV (2). To evaluate the suspected increase in surgical radicality, a matched pair analysis was performed with 8 patients undergoing LT for hilar cholangiocarcinoma without partial pancreatoduodenectomy. The 1-, 5-, and 10-year patient survival rates after EBDR were 63%, 38%, and 38%, respectively. Twelve patients died: 2 died because of postoperative complications, 8 died because of tumor recurrence, and 2 died while recurrence-free more than 10 years after transplantation. Among the 6 stage I patients, only 1 developed tumor recurrence, but 2 died because of postoperative complications. The following factors showed a trend toward inferior survival: distant metastases, positive lymph nodes, high carbohydrate antigen 19-9 levels, and preoperative percutaneous transhepatic cholangiodrainage. When all lymph node-negative patients were considered after the exclusion of perioperative deaths, 10-year survival was 56%. In conclusion, the overall long-term survival was relatively low in our inhomogeneous cohort but favorable in patients without metastases. However, because of the increased perioperative mortality, EBDR is not recommended as a standard procedure for hilar cholangiocarcinoma instead of LT alone. To further improve the results, other approaches such as (neo)adjuvant therapy have to be increasingly investigated.