A 13-year-old female patient is presented who had hypofibrinogenemia diagnosed as von Willebrand disease at 5 years of age at another hospital. She was admitted to the department of pediatric hematology with a severe headache, vomiting, and progressive right flaccid hemiplegia and lethargy. Contrast-enhanced computed tomography scan showed subdural hematoma in posterior parietal region of the brain and impending cerebellar herniation. She was given fresh-frozen plasma (FFP) and then activated factor VII (rFVIIa), 80 microg/kg was infused for replacement of von Willebrand factor. The subdural hematoma was emergently drained. The results of coagulation tests before infusion of FFP and rFVIIa revealed hypofibrinogenemia, and FFP was given every 48 hours. The patient recovered dramatically in a few days. Five days after rFVIIa infusion, a magnetic resonance angiography-proven right middle cerebral arterial thrombosis developed. It is an interesting point of discussion whether the middle cerebral arterial thrombosis was provoked as a consequence of rFVIIa and FFP infusion.