This retrospective study in 79 surgically treated patients with a proximal bile duct carcinoma revealed 12 patients with a median age of 59.5 years (range 21-73 years) who survived more than 5 years. These 12 patients were analyzed to identify specific patient characteristics for long-term survival. Fifteen patients died from postoperative complications and were excluded from this survival analysis. In relation with preoperative Bismuth classification, there were 3 (20%) long-term survivors of 15 patients with type I tumors and 9 (35%) long-term survivors of 26 patients with type II tumors. In the group of type III and IV tumors, there were no long-term survivors. Concerning the type of resection, 9 of 51 (18%) patients had long-term survival after local resection and 3 of 13 (23%) patients after local resection combined with hemihepatectomy. Complete tumor-free surgical specimen margins were found in only 4 of 64 cases (6%), among which only one patient survived more than 5 years. Negative proximal bile duct margins, absence of multifocality, and diploid tumors showed a significant correlation with long-term survival. There was no significant correlation between long-term survival and postoperative radiotherapy. Of the 12 long-term survivors, 5 died after 5 years: 2 had developed metastases and 1 a local recurrence; the other 2 died of a metastasis of an ovarian adenocarcinoma and cachexia, respectively. The remaining seven patients were still alive at the completion of this study. The mean survival of the 64 patients analyzed in this study (in which hospital mortality was excluded) was 33.7 months, with a median survival of 18.8 months. In conclusion, the preoperative Bismuth classification of the tumor, absence of multifocality, diploid-type tumors, and negative proximal bile duct margins at histopathologic examination were the only significant prognostic factors for long-term survival.