Key Clinical MessageSpontaneous spinal epidural hematoma (SSEH) rarely occurs. Without early diagnosis, SSEH can lead to the acute onset of neurologic deficits. We report the case of a 65‐year‐old male with diabetes mellitus who was admitted to our emergency department with a chief complaint of sharp and severe pain in the left scapula and behind the sternum. He was misdiagnosed with cardiovascular disease until the onset of progressive bilateral paraplegia and lower limb numbness. Magnetic resonance imaging revealed a ventral thoracic SSEH. Surgical treatment to remove epidural hematoma and laminectomy for decompression were performed. Except for urine retention, bilateral lower limb paraplegia and numbness were alleviated postoperatively. Due to the high risk of poor neurological outcomes without treatment or with delayed intervention, timely surgical evacuation of the hematoma and hemostasis are recommended to ensure favorable neurological outcomes.