Spontaneous spinal epidural hematoma (SSEH) is rare in children, especially in infants, in whom only 12 cases have been reported. Because of the nonspecificity of presenting symptoms in children, the diagnosis may be delayed. We report herein a case of SSEH in a 20-month-old girl who initially presented with neck pain, and developed lower extremity motor weakness and symptoms of neurogenic bladder 2 weeks prior to admission. The magnetic resonance imaging showed an epidural mass lesion extending from C7 to T4, and the spinal cord was severely compressed by the mass. After emergency decompressive surgery the neurologic function was improved immediately. Two months after surgery, the neurological status was normal with achievement of spontaneous voiding. We suggest that surgical intervention can provide excellent prognosis in case of SSEH in infants, even if surgery delayed.