Intracranial hemorrhage in patients with hemophilia is associated with high mortality and sequelae. We report the case of 50-year-old man with Hemophilia A, who presented with spontaneous acute subdural hematoma and underwent craniotomy for clot evacuation. The patient received Factor VIII infusions perioperatively along with other measures to decrease blood loss. The patient presented with signs of high intracranial tension and received 3% saline intraoperatively and postoperatively to prevent brain edema. Recommendations for perioperative preparation and management of hemophilia, especially in the setting of emergency major surgery are reviewed.