Esophageal schwannoma is very rare neoplasm, which is difficult to diagnose by endoscopy or radiologic evaluations. The diagnosis is not confirmed until immunohistochemical tests are performed after a surgeon has resected the lesion. We present the case of a 65-year-old male patient with an esophageal schwannoma having a palpable neck mass and severe dysphagia. The postoperative pathological findings revealed a strong immunoactivity to S-100 protein but negative activity to smooth muscle actin and C-kit. These results support the characteristics of schwannoma in the tumor.