Spinal dural arteriovenous fistulas (SDAVF) are a rare pathologic entity with a diverse and often misleading clinical presentation. While digital subtraction spinal angiography remains the gold standard, recent advances in noninvasive vascular imaging have improved the diagnosis of SDAVF. As this condition can result in permanent spinal cord injury, all patients require treatment, which consists of surgical or endovascular occlusion of the fistula. Failure to recognize and treat SDAVF in a timely fashion can result in irreversible neurologic disability, including myelopathy, lower extremity weakness and bowel, bladder and sexual dysfunction. This article reviews the clinical features, pathogenesis, radiographic features and current treatment strategies for these complex lesions.