Dural arteriovenous fistulas (DAVFs) may occur anywhere there is a dural or meningeal covering around the brain or spinal cord. Clinical manifestations are mostly related to venous hypertension, and may be protean, acute or chronic, ranging from minor to severe, from non-disabling tinnitus to focal neurological deficits, seizures, hydrocephalus, psychiatric disturbances, and developmental delay in pediatric patients. Although low-grade lesions may have a benign course and spontaneous involution may occasionally occur (i.e. cavernous sinus DAVFs), the risk of hemorrhage is considerable in high grade lesions. Angiographic features of DAVFs have been clarified since the 1970s when venous drainage pattern was clearly identified as the most significant risk predictor and as a major determinant of success or failure of treatment. The mainstay of therapy is interruption of arteriovenous shunting, which has traditionally been accomplished surgically. Currently, endovascular therapy is generally considered the first line of treatment, allowing elimination of the lesion in most patients, with surgery and stereotactic radiosurgery reserved for complex situations. This review discusses major aspects of DAVFs, including grading systems, clinical presentation, diagnostic evaluation, various issues impacting endovascular therapy, and pathophysiology.