We describe a 24-year-old woman who presented with twice previously unverified subarachnoid hemorrhages from the conus medullaris spinal arteriovenous malformation with Parkes-Weber-syndrome. Spinal MRI examination is considered to be necessary for the diagnosis of Klippel-Trenaunay-Weber syndrome. For diagnosis of the spinal cord arteriovenous malformation, it is indispensable to search carefully for the presence of accompanying lesions. Transarterial glue embolizationis effective for the management of the spinal vascular lesion.