Spinal vascular malformations are a rare heterogeneous entity of spinal lesions with abnormal blood vessels. They may occur extra- and intradurally, peri- and intramedullary, with confined or complex configuration, and at various levels of the spine. Associated clinical symptoms are extremely variable and may range from diffuse pain, to progressive myelopathy, and even to acute hemorrhage with severe neurological deficits. Often, the patients present with significant spinal cord injury before the spinal arteriovenous lesions are diagnosed. Early recognition of the pathology is essential to cease potential disease progression and neurological deterioration. Therefore, the purpose of this review was to describe the current findings in spinal arteriovenous lesions focusing on the timing of treatment and treatment modality.