We report a case of small cell carcinoma of extrahepatic bile duct presenting with jaundice and hemobilia. A 59-year-old woman was admitted due to right upper quadrant pain and jaundice. An abdominal computed tomography revealed a 2 cm sized mass in the extrahepatic bile duct. Endoscopic retrograde cholangiopancreatography revealed bloody discharge coming out of the papillary orifice in endoscopic view and a dilated extrahepatic bile duct with multiple irregular filling defects in cholangiogram. A coronal T2-weighted image revealed a hyperintense mass at extrahepatic bile duct. Laparotomy was performed, and pathologic examination of resected specimen showed tumor cells having round to oval nuclei with coarsely granular chromatin and scanty cytoplasm, which were immunoreactive for synaptophysin and chromogranin A, compatible with the diagnosis of small cell carcinoma. The small cell carcinoma of bile duct, despite its rarity, should be considered in differential diagnosis of the causes for obstructive jaundice and hemobilia.