Spinal vascular malformations include various entities such as spinal arteriovenous malformations (AVM) and represent a rare disease. Patients may present with acute or progressive sensorimotor deficits and/or nonspecific symptoms such as hypoesthesia or paresthesia, diffuse back and muscle pain, or feelings of weakness. When spinal arteriovenous malformation is suspected, magnetic resonance imaging (MRI) is the first diagnostic step to rule out other differential diagnoses, followed by a selective spinal angiography. Therapeutic options include endovascular therapy by embolization and/or surgical resection. If spinal AVM is treated properly, a significant reduction in symptoms can be achieved.