Epithelioid hemangioendothelioma, an aggressive vascular tumor has the rarity of morbidity that arises in the spine. There were few cases reported in literatures in recent years, and little was known about this disease. A review study of the patient files in our constitutions between 1996 and 2006 showed that five patients were treated for spinal epithelioid hemangioendothelioma. Although only five patients, this study attempts to bring more informations about this rare lesion. This patient group included two males and three females. The lesions located in the cervical (case 1) or thoracic (case 2-4) or lumbar spine (case 5). Treatments included: laminectomy and cytoreductive surgery followed by external beam irradiation (one patient), expanded resection in piece meal with postoperative external beam irradiation (three patients), and total en bloc resection alone (one patient). Reconstruction of the spinal stability was performed in four patients. Follow-up period ranged from 25 to 72 months, averaged 47.4 months. The neurologic function of patients got a satisfactory progress except the paraplegic patient at diagnosis. The patient who received laminectomy and cytoreductive surgery followed by external beam irradiation still presented with tumor local progress, metastasis, and she died at 34 months after operation. No local recurrence or distant metastasis was detected in the other four patients. Epithelioid hemangioendothelioma of the spine is so rare in clinic as a primary aggressive vascular tumor. Based on our experience, a valid expanded resection of the tumor with adjunct radiation therapy or total en bloc excision may present with acceptable results.