Background Factor VII is essential for coagulation activation by the extrinsic pathway. Hemorrhages of the central nervous system in patients with congenital factor VII deficiency seem to have a higher incidence compared with other congenital coagulopathies. The purpose of this paper is to report two rare cases of subarachnoid hemorrhage and factor VII deficiency. Case Description Two cases of women affected by a congenital deficiency of factor VII and subarachnoid hemorrhage are reported. Diagnosis was obtained by cerebral computer tomography; cerebral panangiography was normal. Complete coagulation studies were performed showing prothrombin time prolongation and factor VII deficiency. In one patient, family studies revealed the existence of a similar coagulation disorder. Conclusions We suggest routine coagulation studies in all patients with subarachnoid hemorrhage. Determination of factor VII activity might be performed in patients with normal activated partial thromboplastin time and prolonged prothrombin time.