A 1-year-old male with hemophilia A (i.e., severe deficit of factor VIII) presented with the acute and spontaneous onset of severe quadriparesis. Laboratory tests revealed a low hemoglobin (8.5 g/dL), low hematocrit (25.6%), a mildly elevated international normalized ratio (1.34) and partial thromboplastin time (37 s), with reduced prothrombin activity (65 s). The holo-spine magnetic resonance (MR) with contrast showed a large cervicothoracic posterior epidural compressive lesion extending from C3 to L1 that was consistent with an epidural bleed; it was hypointense on T1, and hyperintense on T2-weighted images [ and ]. An urgent C5-T10 right hemilaminectomy was performed along with the simultaneous administration/infusion of VIII replacement. The 48-h postoperative cervicothoracic magnetic resonance imaging documented complete epidural hematoma removal with no increased signal in the cord (i.e., consistent with the absence of a significant ischemic injury) []. Postoperatively, the factor VIII deficit was confirmed and he was treated with continued VIII factor infusions (i.e., 500 UI twice a day, for a total of 6 days). Five years later, he exhibited no residual deficit and demonstrated no spinal deformity.