Benign tumors of the esophagus are rare, and almost all of them are leiomyomas. Esophageal schwannoma is extremely rare. We report a case of esophageal schwannoma in a 54-year-old Taiwanese man who was admitted due to dysphagia. Barium esophagogram revealed a protrusive smooth-marginated filling defect of about 3.6 cm in diameter with intact mucosa over the lower third of the esophagus. The submucosal tumor was removed via left thoracotomy with enucleation. There was no anatomic relationship between the tumor and the vagal nerve trunk. Histopathologic examination revealed interwined bundles of spindle cells with spiral-like proliferation. Immunohistochemical study was positive for S-100 protein. Esophageal schwannoma was diagnosed. The patient had no postoperative complications, and was healthy and free of tumor recurrence 16 months after operation.