Endocrine tumors of the extrahepatic bile ducts are an extremely rare clinical entity with only very few cases being reported to date. Two additional cases of extrahepatic endocrine tumors are presented and a review of the literature is analyzed to provide a comprehensive data collection. Thirty-four cases of extrahepatic endocrine tumor, including our 2 cases, have been reported; 16 (47%) being carcinoma. The mean age of presentation was 48.2 years with a male/female ratio of 0.36. Jaundice, the most common symptom, was found in 63% of the patients. The common bile duct and the hilar region were the two most frequent anatomic locations. The mean size of resectable tumors was 2.37cm (0.2- 5.5cm). Metastases were present in 11 patients (32.35%) and local invasion was found only in 6 patients (17.6%). Curative surgery was realized in the majority of the patients. Long-term disease-free survival as long as 20 years was reported when surgery was curative. Endocrine tumors of the extrahepatic duct are exceptional and represent 0.2% of all cancers of these anatomic sites. Curative resection is the most important prognostic factor. Therefore, aggressive surgical therapy with curative intention offers the only chance for cure and has to be considered whenever possible.