Spinal cord vascular malformations are often a cause of spinal myelopathy. Endovascular surgical approaches to spinal cord vascular malformations have become an important adjunct and often the primary treatment of these disorders. Spinal cord vascular malformations may be divided into several categories based on their anatomy and location. They may present with a wide range of symptoms far removed from the vascular pathology. Early recognition and treatment can effect a better outcome and often reverse presenting neurologic deficits. Emerging magnetic resonance imaging techniques allow visualization of flow voids, some vascular anatomy, and intramedullary pathology and are a good screening modality. However, selective diagnostic spinal angiography remains the study of choice to diagnose and elaborate on the anatomy and potential treatment protocols. Rapidly evolving endovascular techniques and technology are revolutionizing the treatment of vascular pathology in the central nervous system, either as an adjunct to traditional neurosurgical techniques or as definitive therapy. We review the diagnosis and treatment options for spinal cord vascular malformations.