We herein report the case of a 52-year-old woman who has mediastinal ganglioneuroma and retroperitoneal pheochromocytoma with different histologic entity. The patient first visited our hospital for surgical treatment of superior-posterior mediastinal tumor, which was successfully removed by thoracoscopic approach to reveal a benign ganglioneuroma. A retroperitoneal tumor was incidentally found postoperatively that was removed using a laparoscopic procedure. The retroperitoneal tumor was a nonfunctioning pheochromocytoma, which was different from the mediastinal neurogenic tumor. This association, although rare, should be recognized for management of neurogenic tumors in the thorax.