Foix and Alajouanine first described the syndrome in two young men with spinal arteriovenous malformation. It was initially thought to be a subacute myelopathy produced by a thrombotic process of the spinal cord that ultimately caused death. In this paper, we report for the first time, the occurrence of intramedullary hemorrhage after a normal vaginal delivery in a 32-year-old primigravida with Foix-Alajouanine syndrome. During pregnancy the patient received subcutaneous heparin, 0.6 ml twice a day until delivery, for prevention of deep vein thrombosis. Our data, for the first time, document objectively the risk of hemorrhagic transformation (presenting with acute compressive myelopathy) posed by normal vaginal delivery in patients with Foix-Alajouanine syndrome.