Objective To assess the long-term survival and prognosfie factors in a series of patients with distal bile duet carcinoma. Methods A retrospective clinical analysis was made on 76 cases of distal bile duct cancer who were admitted into our hospital from January 1996 to December 2006. Clinicopathologic factors with possible prognostic significanees were selected and analyzed. Survival was calculated with the Kaplan-Meier method. A multivariate analysis of these individuals was performed using the Cox proportional Hazards Model. Results There were 46 males and 30 females. The age ranged from 21 to 88 years with a mean of 65.21 patients received palliative surgery including, bypass procedure, intraoperative biliary stenting, or percutaneous transhepatie biliary drainage. Radical resection was performed on 42 cases and the 1-, 3- and 5-yeur survival rates were 88.0%, 41.3% and 29.2% respectively. 38 cases died of liver metastasis or recurrence. In multivariate analysis, surgical procedure (P = 0.006) and liver metastasis (P = 0.008), but not sex, age, invasion of pancreas, invasion of duodenum, diameter or differentiation of tumor, were significant independent prognostic factors. Conclusions Radical resection is only curative treatment modality. Prevention on postoperative liver metastasis is essential for improving survival.