Mucoepidermoid carcinoma of the bile duct is an extremely rare tumor. Seventeen cases originating from intrahepatic bile duct and 2 cases from common hepatic duct have been reported in the English literature. Mucoepidermoid carcinoma arising from the common bile duct has not been previously reported. A 68 year-old man was admitted due to obstructive jaundice. Computed tomography showed a malignant tumor of the common bile duct located in the intrapancreatic segment. Filling defects of the distal common bile duct was seen on endoscopic retrograde cholangiogram. Under the impression of bile duct cancer, pylorus-preserving pancreatoduodenectomy was performed. Histologic diagnosis of the resected specimen was mucoepidermoid carcinoma of the common bile duct. After surgery, the patient received concurrent chemoradiotherapy, and planned to receive additional chemotherapy. We herein report on a first case of primary mucoepidermoid carcinoma of the common bile duct, and review the literature.