Esophageal schwannoma is a very rare submucosal tumor. We report successful management of esophageal schwannoma in a 41-year-old man who complained of progressively worsening dysphagia. A huge submucosal tumor was found via endoscopy and a chest computed tomography scan. Esophagectomy was performed with no post-operative complications. Post-operative immunohistochemistry staining showed a positive result for S-100 and negative results for c-kit and CD34. The post-operative mild dysphagia persisted, and the follow-up endoscopic findings revealed anastomosis site stenosis. Approximately 2 months later, we performed endoscopic balloon dilatation. We report herein a case of esophageal schwannoma with reviews.