Dural arteriovenous fistula (dAVF) of the foramen magnum (FM) region is rare. Moreover, the terminology of dAVF is very confusing in this region. In the narrow sense, the FM dAVF is the non-sinus-type dAVF with direct venous reflux to the medulla oblongata or spinal cord via the bridging veins (BVs) of the FM. Previous literature was systematically reviewed to investigate the clinical characteristics, angioarchitecture, and effective treatment of the FM dAVF. From the literature review, almost all the feeders of FM dAVF were dural branches. Spinal pial arteries were rarely involved as the feeder. All lesions had venous reflux to the medulla oblongata via medullary BVs. The FM dAVF is characterized by a significant male predominance and a high incidence of aggressive symptoms. The most common symptom is congestive myelopathy, followed by hemorrhage. The FM dAVF differs from the craniocervical junction (CCJ) arteriovenous fistula (AVF) and is similar to the thoracolumbar spinal dAVF. Direct surgery for the FM dAVF is effective and safe. Endovascular treatment for the FM dAVF may be more effective and has lower complication rates than that for the CCJ AVF.