Abstract : Spinal epidural hematoma (SEH) is a rare neurosurgical emergency in respect of motor and sensory loss and is an extremely rare complication in hemophilia. Treatment options should be carefully considered for such rare and difficult cases. We report three cases of SEH in severe hemophilia A, who had different treatment and resulted in various prognoses. All of patients had developed SEH spontaneously and had been managed either conservatively or surgically. Their clinical course and prognosis were various under the different treatment strategies. The SEHs, all of which developed spontaneously, were identified by magnetic resonance imaging (MRI). An eight-month-old boy, who was presented with non-traumatic acute-onset irritability and torticollis, was found to have SEH at the level of C1-S1 on MRI and then diagnosed as having hemophilia. The other two patients had neck pain as their initial symptom, while their MRI scans identified an SEH extending from C1 to T6 and from C3 to C5, respectively. The treatment intervals from the onset of the initial symptom to the clotting factor administration for these 3 patients were 17 hours, 27 hours, and 36 hours, respectively. The coagulation factor concentrates to reach 100% factor level or adequate doses of bypassing agents were administered. Conservatively managed 2 patients were fully recovered within 2 months in spite of transient FVIII antibody development after coagulation factor treatment in one pediatric patient. The other patient, who presented with progressive neurologic abnormalities at the time of diagnosis, had to undergo neurosurgery and he remained as non-spontaneous breathing and quadriplegia, post operatively. Early diagnosis and immediate aggressive replacement therapy are crucial in the management of SEH. Owing to high morbidity and mortality, it is imperative for clinicians to decide on optimal management for such SEH cases. Preferences between conservatively or surgically, should be considered carefully for such SEH cases in accordance with a patient’s condition, although the best strategies for good prognosis are still being debated.