After we encountered one case of cystic duct carcinoma out of 110 cases of gallbladder carcinoma treated from 1976 to 1991, we reviewed the 14 treated Japanese cases, including our own, as well as the 18 Western cases hitherto reported in order to examine the clinical features of carcinoma of the cystic duct and to evaluate the various options for treatment. The patients' ages were similarly distributed between the Japanese and Western patients with a mean age of 62 years. Males were predominantly affected in the western countries with a ratio of male to female of 3.5:1; the ratio was similar in Japan. Gallstones were present in six of 18 Western cases and in two of 14 Japanese cases; both groups had a much lower rate of accompanying gallstones than did gallbladder carcinoma patients. Despite the depth of invasion, the prognosis of carcinoma of the cystic duct was relatively good, mainly owing to the absence of either lymph node metastases or distant metastasis. Although the lymph nodes and bile duct are not usually involved in carcinoma of the cystic duct, cholecystectomy, either with or without lymph node dissection, usually produced an unsatisfactory outcome, possibly due to bile duct invasion and perineural invasion. Thus, combined resection of the gallbladder and bile duct with lymph node dissection is the choice of treatment for carcinoma of the cystic duct.