Background Factor VII is essential for coagulation activation by the e
xtrinsic pathway. Hemorrhages of the central nervous system in patient
s with congenital factor VII deficiency seem to have a higher incidenc
e compared with other congenital coagulopathies. The purpose of this p
aper is to report two rare cases of subarachnoid hemorrhage and factor
VII deficiency. Case Description Two cases of women affected by a con
genital deficiency of factor VII and subarachnoid hemorrhage are repor
ted. 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 o
ne patient, family studies revealed the existence of a similar coagula
tion disorder. Conclusions We suggest routine coagulation studies in a
ll patients with subarachnoid hemorrhage. Determination of factor VII
activity might be performed in patients with normal activated partial
thromboplastin time and prolonged prothrombin time.