A carcinoid tumor of the bile duct represents between 0.2 and 2% of all gastrointestinal carcinoids, most of which are located in the gallbladder or in the ampulla of Vater. A carcinoid tumor of the extrahepatic bile duct is extremely rare. A 43-year-old man presented with epigastric discomfort for several months. An abdominal ultrasound revealed a mass of the common bile duct. An endoscopic retrograde cholangiopancreatographic (ERCP) examination showed a 1.5 cm sized filling defect in the distal common bile duct and marked dilatation of the common bile duct and intrahepatic bile ducts. A 7 F endoprosthesis was put in place during the examination. The histological finding following an intralesional biopsy was a carcinoid tumor. We performed a pylorus preserving pancreaticoduodenectomy. The final pathological diagnosis was a well-differentiated carcinoid tumor of a malignant nature. (Korean J Gastrointest Endosc 2008; 36:117-121)