A case of esophageal cancer associated with the right aortic arch in a 62-year-old Japanese male is described herein. Endoscopy performed after an initial complaint of dysphagia revealed cancer at the upper third of the esophagus. The right aortic arch was located by routine chest X-ray and an irregularly-shaped tumor defect and a shadow defect due to right aortic arch were detected on the right side of the thoracic esophagus by barium study. Computed tomography and magnetic resonance imaging offered useful information for surgical procedure. The patient underwent a subtotal esophagectomy and regional lymphadenectomy though a left thoracotomy. The clinical features of cases previously reported in Japan are reviewed following the presentation of this case.