Situs inversus, a left-to-right transposition of the normally asymmetrical organs of the body, is a rare anomaly that occurs in 1:5,000-1:10,000 adults. In the total form, the thoracic organs, as well as the abdominal organs, are completely reversed in a "mirror image" of their normal arrangement. Herein, we present a case of an adenocarcinoma of the proximal bile duct in a 68-year-old man with total situs inversus. Without any technical problems, he successfully underwent a bile-duct resection and bilioenteric anastomosis. He did well postoperatively, and after one course of chemotherapy, was discharged on postoperative day 12. A review of the international literature on this subject is presented.