Right aortic arch is a rare abnormality. Esophageal cancer associated with a vascular ring is even more rare. We describe a patient with right aortic arch who had previously undergone a graft replacement of descending aorta for aneurysmal rupture followed by subsequent surgery for advanced esophageal cancer. Preoperative three-dimensional computed tomography revealed mirror image branching type right aortic arch and Kommerell's diverticulum of the descending aorta. The patient underwent total esophagectomy with laryngopharyngectomy and regional lymphadenectomy via a left thoracotomy. A permanent tracheal fistula was constructed. Postoperative chemo-radiotherapy was applied. The patient was discharged from hospital and is doing well. Early division of the ligamentum arteriosum with careful management of the aortic diverticulum led to release of the esophagus from the vascular ring that rendered the following procedures safe and afforded a good operative view.