Introduction & Objective: Carcinoid tumors are rare tumors of the gastrointestinal tract, and very rare in extra hepatic bile ducts. Only 42 cases have been reported. The similarity of symptoms and sign of carcinoid tumors and common causes of obstructive jaundice, often leads to delay in diagnosis and treatment.Here we introduce a rare case, along with the literature review and discussion of reported cases.Materials & Methods: A 25 years old man presented with right upper quadrant abdominal pain and jaundice for 2.5 years period and progressed gradually. Physical exam was negative except for tachycardia and jaundice. Abdomen was soft and the liver and gall bladder were not palpable. There were no abdominal mass and tenderness. Total Billirubin was 8.9 mgr/dl, Direct Billirubin, 7.3 mgr/dl, AST and ALT were subsequently 90 I.U/lit, 105 I.U/lit, and alkalin phosphatase was 545 I.U/lit.In ultrasonography, billiary tracts were dilated. In ERCP showed, dilation of intrahepatic bile ducts, common hepatic duct (CHD), and proximal part of common bile duct (CBD). With the diagnosis of cholangitis and the probable stricture due to cholangio- carcinoma, the patient scheduled for surgery. A hard, multi lobulated, red mass, 3×3×4.5 cm in diameter superior to first portion of duodenum was detected. The tumor had extended to the junction of common hepatic and cystic ducts, and had caused partial occlusion of the cystic duct. CBD had passed through the mass and was not dissectible from it. Further dissection showed that the mass surrounded CBD completely, making it nonfeasible for being dissected. Resection of tumor and reconstruction by hepatico - jejunostomy was performed. Two weeks following resection of tumor the level of hepatic enzyms and billirubin decreased to normal range. The level of 5-HIAA was also normal. The patient was followed up for 16 months with normal general condition.Conclusions: Carcinoid tumors are neuroendocrine tumors with special histological, clinical and biological characteristics and grow very slowly. Despite the non-specific symptoms in most patients and the absence of specific signs, this neoplasm must always be considered as differential diagnosis. In obstructive jaundice when the definite diagnosis can not be made with non-invasive methods like ultarsonography and laboratory tests, other methods like ERCP and the measurement of serotonin end products needs to be considered.