Extrahepatic bile duct tumors, 80 per cent of which are adenocarcinomas, are rare neoplasms accounting for less than two per cent of all cancers. Carcinoid tumor of the extrahepatic bile ducts is a reportable lesion, with only approximately 50 cases described in the literature since 1959. We present a case of a primary extrahepatic bile duct carcinoid tumor resected for cure with the longest reported follow-up time (11 years) after surgery. We also summarize the existing literature with regard to this rare tumor. Our case lends strong support to the notion that extrahepatic biliary carcinoids are generally indolent lesions that, if aggressively resected, can result in excellent long-term survival. Complete excision with clear margins seems to provide the best chance of obtaining long-term survival and cure.