Spontaneous spinal epidural hematoma (SSEH) is a rare space-occupying disease. The pathogenesis of SSEH is unclear, but anticoagulant therapy, arteriovenous malformation, coagulopathy, tumors and infection are risk factors. Ventral SSEH is even more rare, as the ventral sac is fixed firmly to the posterior longitudinal ligament. The authors report a very rare case, a 16-year-old previously healthy female who had repeated relapse of cervico-thoracic SSEH. This occurred three times in the ventral epidural space with subsequent syringomyelia, without structural abnormality and coagulopathy. The patient underwent conservative care twice, and two operations. The diagnosis was made by magnetic resonance imaging. In rapidly progressing SSEH, the clinician should make the diagnosis as early as possible to enable spinal decompression surgery.