Leiomyomas, originating in the bile duct, are very rare, and only few cases have been reported in the literature. We experienced a case of leiomyoma of the distal common bile duct, mimicking bile duct cancer. A 39-year-old woman presented with intermittent jaundice and general weakness for three months. Clinical profiles showed obstructive jaundice, and the abdominal computed tomography and cholangiography revealed diffuse bile duct dilatation with distal common bile duct stricture. A pylorus-preserving pancreaticoduodenectomy was performed and the pathologic specimen disclosed leiomyoma of the common bile duct accompanying severe fibrosis. This is the first case of leiomyoma in the bile duct reported in Korea.