The aim of this study was to delineate clinical features and prognosis of cancer of the gallbladder associated with anomalous junction of the pancreatobiliary duct system without bile duct dilatation, and to determine methods for managing the disease. A retrospective study of seven patients is presented. A further 27 cases from the Japanese literature were reviewed retrospectively with regard to method of treatment and prognosis. In 11 of 18 patients in whom staging was known the tumour was stage V, representing advanced disease. In seven of 34 cases curative operation was performed; only two patients survived for > 3 years. This poor outcome was due largely to delayed diagnosis of cancer of the gallbladder. Prophylactic cholecystectomy is recommended in patients with this anomalous junction without bile duct dilatation or a malignant lesion in the gallbladder, because of the high incidence of cancer of the biliary tract.