A case of spontaneous hematomyelia was reported with a review of the literature. A 39-year-old woman, with sudden onset of pain in her left neck and arm 13 days previously, presented with a progressive flaccid tetraplegia below C5, dissociated sensory loss from C7 through Th4 dermatome and a minimal deep sensory loss in her right leg. Myelography revealed a symmetrical enlargement of the spinal cord at the leve of C5, C6 and C7. Laminectomy was performed and intramedullary blood clot, which was located in the region of C6 and C7, was evacuated. However, there were no vascular anomalies not only in macroscopic findings but also in histological examination. The postoperative course was uneventful and the patient gradually improved in her motility. At 5 month' follow-up examination she continued to do well with a minimal sensory loss of all modalities below the level of C7. Report of 16 patients with spontaneous hematomyelia including our case have been reviewed. Incidence of spontaneous hematomyelia without vascular anomalies or hemorrhagic diathesis very rare, being found in only 4 cases. In cases with good outcome the intramedullary hematoma was found to be located below the lower cervical region and limited within 2 spinal segments in its extent. Good postoperative results were obtained in 5 out of 7 patients who received surgical intervention, suggesting an essential importance of early diagnosis and surgery.