We describe the clinical presentation, radiographic findings, management, and outcome of nontraumatic spinal epidural hematoma in a 10-month-old male infant with severe hemophilia (<1% activity). This patient presented with torticollis, and the differential diagnosis included intramuscular hemorrhage, retropharyngeal abscess, muscle spasm, and epidural hematoma. A computed tomography scan revealed extensive spinal epidural hematoma from C1-L4. Because of prompt diagnosis, this infant was able to be managed conservatively with factor VIII and did not require surgical intervention. Unlike other cases previously published, this case demonstrates how prompt recognition, diagnosis, and treatment can prevent the development of neurological deficits.