Purpose. Disease-specific survival (DSS) for proximal bile duct cancer has been reported to be worse than for carcinoma of the distal duct. Methods. Review of two prospectively maintained databases identified 204 patients who underwent resection for extrahepatic cholangiocarcinoma (proximal: n=106, 52%; distal: n=98, 48%) between December 1987 and December 2005. Patient, tumor, and treatment-related variables were reviewed. Analyses were performed to compare tumor presentation, treatment, and DSS between patients with resected proximal and distal lesions. Results. Median follow-up for the 204 resected patients was 24 months (range 1–165 months) and 56 months for those alive at last follow-up. Combined liver/bile duct resection was performed in 82% of patients with proximal lesions, and pancreaticoduodenectomy was performed in 92% of patients with distal lesions. Patients experienced similar postoperative length of stay (median: proximal, 13 days vs. distal, 13 days; p=0.64) and operative mortality (30-day: proximal, 4% vs. distal, 3%; p=1.0, Fishers). Margin positive rates were similar (proximal, 23% vs. distal, 15%; p=0.20). Estimated five-year DSS for all patients was 35%. Tumor location (proximal vs. distal) was not associated with five-year estimated DSS (proximal, 29% vs. distal, 43%; p=0.44). Factors associated with five-year DSS included stage at presentation (node negative, 42% vs. node positive, 22%; p=<0.001), differentiation (papillary, 53% vs. non-papillary, 27%; p=0.01), and margin status (margin negative 42% vs. margin positive 27%; p<0.001). Conclusions. These results suggest that patients with resected proximal and distal cholangiocarcinoma will experience similar operative outcomes and DSS.