We report herein the case of a 37-year-old woman found to have double cancer of the gallbladder and common bile duct associated with an anomalous pancreaticobiliary ductal junction (APBDJ) without a choledochal cyst (CC). Abdominal ultrasonography showed an isoechoic mass in the gallbladder, and percutaneous transhepatic biliary drainage tubography revealed incomplete obstruction in the upper portion of the common bile duct and APBDJ. The patient underwent cholecystectomy, partial hepatic resection, pancreatoduodenectomy, and portal vein reconstruction. Pathological examination of the tumors from the gallbladder and bile duct revealed papillary carcinoma and poorly differentiated adenocarcinoma, respectively, and direct continuity was not observed between the tumors. A review of the literature on six cases of multiple primary carcinoma of the biliary tract associated with APBDJ without CC is presented following this case report. Double cancer of the biliary tract was found synchronously in five patients and metachronously in one. Gallbladder cancer showed subserosal invasion in four patients, while bile duct cancer invaded the pancreas in one patient and reached the serosa in two patients. Considering the potential for cancer to arise in the biliary tract and the difficulties associated with monitoring it, cholecystectomy and resection of the extrahepatic common bile duct may be the most appropriate treatment for patients with an APBDJ without a CC.