A case of malignant esophageal schwannoma is reported. A 54-year-old man consulted for a 1-year history of dysphagia. Investigations revealed a tumor of the distal esophagus, with involvement of the cardia, and were suspicious for metastatic mediastinal nodes. Ivor-Lewis esophagectomy with gastric-tube reconstruction was performed, with favorable outcome. Histological examination revealed esophageal sarcoma in a Barrett's esophagus. Periesophageal nodes had metastatic involvement. Immunohistochemical study was positive for S100 and vimentin and was negative for CD117, compatible with a diagnosis of esophageal schwannoma. We discuss this rare disease and its characteristics. This is the second reported case of malignant schwannoma with lymph node metastasis.