A case of traumatic epidural haematoma associated with von Willebrand disease (type 3) is reported. A 32-year-old man fell and hit his frontal region against the floor. CT scan on admission showed acute epidural haematoma of the right frontal region 45 mm in width and 60 ml volume. Considering moderate clinical symptomatology, a possibility of complication in connection with a congenital coagulation disorder and, above all, lack of patient's consent, prophylactic treatment with clotting factor concentrates was undertaken. Complete resorption of epidural haematoma has been obtained. In our opinion, in the presence of relative contraindication to operative intervention, even in cases with a large intracranial haematoma and inborn clotting deficiency prophylactic treatment may be considered. The following conditions must be met before such treatment is undertaken: prompt use of appropriate clotting factors, CT scan, clinical observation as well as possibility of prompt operative intervention in the neurosurgical department must all be available.