A 64-year-old Thai man had a primary mucoepidermoid carcinoma, a variant of the cholangiocarcinoma, of the left lobe of the liver. A 5 x 4 x 3 cm tumor metastasized within the liver and to the porta hepatic and parapancreatic lymph nodes with compression of the head of pancreas and distal part of the common bile duct resulting in obstructive janudice. The patient died within 7 months from massive hemorrhage of a chronic peptic ulcer of the duodenum. Review of the medical literature in the English language disclosed 11 primary mucoepidermoid carcinomas of the bile ducts, including the present instance. Nine tumors were intrahepatic; three were in the right lobe and six in the left lobe. Two neoplasms were extrahepatic; they arose in the common hepatic ducts. There were 6 men and 5 women of 44 to 78 years old; the mean age was 60 years. The size of the tumor ranged from 1.5 to 18 cm in greatest dimension; the average size was 8.4 cm. The tumor metastasized frequently to the regional lymph nodes. Invasion of the portal vein and hepatic artery has also occurred. Ten patients having primary mucoepidermoid carcinoma of the bile duct died within 11 months regardless of treatments. Only one patient was well, 10 months after extensive resection of a 1.5 cm tumor of the common hepatic duct. It is concluded that the prognosis is generally poor for the patient having primary mucoepidermoid carcinoma of the bile duct.