Abstract A rare case of esophageal schwannoma is presented. A 63-year-old woman was admitted to our hospital with a 5-year history of dysphagia. The barium esophagogram showed a protrusive smooth tumor in the upper thoracic esophagus. The tumor was removed through right thoracotomy. There was no anatomical relationship between the tumor and vagal nerve trunk. From pathologic findings and positive immunohistochemical staining for S-100 protein, the diagnosis of esophageal schwannoma was made.