Spontaneous spinal epidural hematoma (SEH) is extremely rare in patients with end-stage renal disease (ESRD). We report a case of a 71-year-old man with ESRD presented with progressive left limb weakness. Magnetic resonance imaging (MRI) revealed spinal cervical SEH involving C3-5 level. The patient received emergent decompression laminectomy with the evacuation of the epidural hematoma.