Background: Cholangiocarcinoma was considered as a dismal disease with very poor prognosis until re- cently. Cholangiocarcinoma is increasingly found due to increased life expectancy. Although surgical and medical management were advanced recently, data on the prognosis, especially extrahepatic cholangio- carcinoma (ECC), were limited. This study aimed to identify clinicopathologic features and prognosis of patients with ECC.Methods: Patients followed up and diagnosed with ECC between January 2014 and December 2016 at a tertiary hospital were included, whereas those with intrahepatic cholangiocarcinoma, gallbladder cancer, and ampullary cancer were excluded. Results: A total of 83 patients were followed after the treatment (49 men and 34 women; median age 73.3 years). Cancer location was classified as distal common bile duct (25 patients), proximal com- mon bile duct (24 patients), common hepatic duct (20 patients), and hilar (14 patients). About 14.5％ of patients had history of another malignant neoplasm, and 24.1％ patients had chronic illness. Surgi- cal resection was performed in 54 patients (65％) and dysplasia was combined in 63％ (34/54). Adjuvant chemotherapy was performed in 54％ (29/54), but only 7 underwent palliative chemotherapy in 29 non- surgical patients. The median overall survival in all patients was 30.9 months. In analyzing the treatment modality, median survival of adjuvant chemotherapy, surgery only, palliative chemotherapy, and support- ive care groups were 42.9, 30.9, 12.0, and 8.9 months, respectively ( P < 0.05). In the Cox regression analy- sis of survival, age, surgical resection, chemotherapy, and comorbidity were significant prognostic factors, and the comorbidity was the only significant prognostic factor in the multivariable analysis (hazard ratio [HR] = 2.80; 95％ CI: 1.32–5.95; P = 0.007). In a subgroup analysis of surgical patients, the presence of dysplasia was a favorable prognostic factor in the multivariable analysis (HR = 0.29; 95％ CI: 0.09–0.91; P = 0.033). Conclusions: The overall survival of patients with ECC was quite high and increased with chemotherapy. Absence of comorbidity, and presence of dysplasia were good prognostic factors.