The case of a 7-year-old boy with a spinal epidural extraosseous Ewing's sarcoma (EES) is presented. He is in complete remission without neurologic deficit 40 months after diagnosis. Another 15 cases were found in the literature and are discussed together with this patient. Twelve of them were male patients. The mean age of the patients was 17.5 years (range, 4 to 47). Symptoms included back pain and/or radicular pain (100%), paresis of one or both legs (83%), sensory disturbances, and bladder and bowel dysfunction. The mean diagnostic delay was 5.8 months. Each patient underwent laminectomy; complete resection of the tumor was impossible in more than 50% of the cases. Most patients received radiation therapy and/or chemotherapy. Four patients suffered from local recurrence, eight from metastases. Ten (63%) patients died, 1 to 48 months (mean, 16) after diagnosis. The differential diagnosis is discussed, including disk herniation and several benign and malignant tumors.