© 2014 Wolters Kluwer Health, Inc. All rights reserved.A 22-year-old man presented with severe head and neck pain, nausea, and vomitting at the emergency department. Computed tomography revealed nonperimesencephalic subarachnoid hemorrhage. Subarachnoid hemorrhage is caused by a spinal glomus type (type 2) intramedullar arteriovenous malformation (AVM) at Th10-12 level. Venous hypertension is considered as the underlying factor of subarachnoid hemorrhage associated with spinal AVM. We aimed to present a spinal glomus type (type 2) intramedullary AVM that presented with nonperimesencephalic subarachnoid hemorrhage accompanied by the literature.