Thirty-six patients with primary Ewing's sarcoma of the spine were diagnosed at the Mayo Clinic between 1951 and 1988. The mean age was 17 years (range, 5-40 years). Neurologic symptoms and signs were seen in 58% of the patients. Forty-seven percent of all patients had an open biopsy of the lesion and underwent a decompressive laminectomy. Three of the four patients with thoracic or thoracolumbar involvement had progressive kyphosis after laminectomy. All patients received radiation therapy in various dosages. Sixteen of the patients were registered in the Intergroup Ewing's Sarcoma Study. Intensive combination chemotherapy was administered to 32 of the patients. Nine patients were free of disease at the final follow-up examination (follow-up ranged from 6 to 184 months). The 5-year survival rate was 33%. The mean survival time was 2.9 years. No significant correlation was found between the location of the tumor in the spine and the length of disease-free survival, overall survival, or incidence of metastatic disease. Patients enrolled in the Intergroup Ewing's Sarcoma Study had significantly better rates of disease-free survival and overall survival.