Only 34 cases of primary cystic duct carcinoma have previously been published in the literature. Most of these cases presented with upper abdominal pain and a palpable mass in the right upper quadrant due to gallbladder hydrops or cholecystitis. We report a case of cystic duct carcinoma with the clinical presentation of obstructive jaundice. The patient was treated by cholecystectomy, resection of the common bile duct and a Roux-en-Y hepaticojejunostomy. An extended lymph node dissection was not performed. Fourteen months after the operation the patient died with local carcinoma recurrence. A literature review comparing clinical signs, surgical treatment, and outcome of 14 Japanese and 21 reported Western cases, including ours, was performed. Extended lymph node dissection in addition to combined resection of the gallbladder and ductus hepaticocholedochus appears to offer a better prognosis and larger survival, including the chance of potential cure.