The authors describe the clinical findings, radiographic appearance and surgical treatment of a spinal epidural hematoma (SEH) associated with spinal fracture in diffuse idiopathic skeletal hyperostosis (DISH). An 81-year-old male patient injured after falling from a 2.5 m tree presented with severe back pain. On plain radiographs and CT images, DISH with anterolateral osteophytes and fused thoracolumbar vertebrae was found along with a T12 fracture. Patient was initially treated with bed rest followed by placement into a body cast. Three weeks later, he presented with incomplete paraplegia of his lower limbs. CT images did not reveal any fracture displacement, but MRI images showed an epidural hematoma compressing the dura mater. The patient was successfully treated by posterior fixation surgery using pedicle screws and rod with vertebroplasty. We aimed to report a unique case of a SEH complicating a spinal fracture in DISH.