Hemophilia A is an X-linked bleeding disorder, caused by deficient or defective blood coagulation factor VIII. The most characteristic symptoms of the severe forms of hemophilia A are joint and muscle bleeds. Intracranial hemorrhage occurs only in 3-10% of the patients. Spinal epidural hematomas are rarely seen. We describe a 13-month-old boy with hemophilia A who was admitted to the hospital because of irritability and unspecified pain for the past two days. There was no history of evident trauma, no fever. Physical investigation showed no skin lesions or hematomas and no obvious cause for the pain. Neurological examination showed a dysphoric toddler, mainly in the fetal position. No neurological abnormalities were found except for a miosis of the right pupil due to a suspected Horner syndrome. Magnetic resonance imaging of the spine showed an extensive epidural hematoma. The boy was successfully treated with intensive replacement therapy during three weeks and did not require surgical intervention. There was a rapid and complete clinical resolution. In conclusion, rare hematomas should be considered and searched for in children with bleeding disorders and not well understood complaints. Early diagnosis is important for the neurological outcome.