Extrahepatic cholangiocarcinoma (EHCC) is rare, often late known, so resection of tumor is seldom in the country. In the USA, the frequence is about 1/100,000. Many tumors are resected, the ratio of 5 year survival is high. Objective: The authors study the epidemilogy, the diagnosic, the treatment and the results. Patients and Method: Retrospectve study all cases of extrahepatic cholangiocarcinoma from 1/1/2010 to 30/12/2013. The authors compare the results with the study on 2004. In 4 years, the authors have 266 cases of extrahepatic cholangiocarcinoma: 120 are Vater cancer and 146, cancer of the CBD and hepatic carefour. Average age 61, male/female 1,2. Jaundice is 85 percent, CA 19.9, 76 percent, adenocarcinoma is majorated. Treatment: 17 Whipple for cancer of Vater, resection of CBD and hepatic carefour (liver) are 4 cases.. 245 cases, stenting, internal or external drainage and chemotherapy. 1 Whipple death due to bleeding. During 24 months, 1 dead due to recurrent cancer and metas tase, another dead due to metastase. Majority of cases have chemotherapy. The cases of stenting, drainage are not survival for 12 months. Discussion: Extrahepatic cholangicarcinoma, male is predominant, average age 61, the same as reported 2005. In the advanced countries, the average age is higher, Jaundice is pedominant. Whipple procedure for Vater cancer is majorated. Resection for cancer of CBD and hepatic carefour is rare. In case of resection, above 80 percent survives for 2 years. The 245 patients with bile drained, no one survival more than 12 nonths. Conclusion: Extrahepatic cholangiocarcinoma is severe, and late diagnosed except cancer of Vater, so only palliative treatment, the survival is in the limit of 12 months. In comparison of the results in a study 2000-2004, the rate of radical operation decreases but the rate of palliative operation increases, especially in internal drainage and stenting.