Inherited factor VII (FVII) deficiency is a rare autosomal-recessive bleeding disorder. There are no clear guidelines regarding therapy in such patients when intracerebral surgery is performed. We report the use of recombinant activated FVII (rFVIIa) for the prophylaxis of bleeding in a female with FVII deficiency (8% of activity) undergoing urgent removal of a right fronto-rolandic intracerebral haematoma secondary to a bleeding from a cavernous angioma. To assist haemostasis during and after surgery, rFVIIa boluses were administered during the procedure and continued every 12 h during 3 days after operation to maintain a prothrombin time <15 s. Using this approach, no abnormal bleeding or thromboembolic complications were observed and rFVIIa appeared safe in this context.