The lateral foramen magnum region is defined as the bilateral occipital area that runs laterally up to the jugular foramen. The critical vasculatures of this region are not completely understood. Dural arteriovenous fistulas that occur in this region are rare and difficult to treat. Therefore, we searched PubMed to identify all relevant previously published English language articles about lateral foramen magnum dural arteriovenous fistulas, and we performed a review of this literature to increase understanding about these fistulas. Four types of dural arteriovenous fistulas occur in the lateral foramen magnum region. These include anterior condylar confluence and anterior condylar vein dural arteriovenous fistulas, posterior condylar canal dural arteriovenous fistulas, marginal sinus dural arteriovenous fistulas, and jugular foramen dural arteriovenous fistulas. These dural arteriovenous fistulas share similar angioarchitectures and clinical characteristics. The clinical presentations of lateral foramen magnum dural arteriovenous fistulas include pulsatile tinnitus, intracranial hemorrhage, myelopathy, orbital symptoms, and cranial nerve palsy. Currently, head computed tomography, computed tomography angiography, magnetic resonance imaging, magnetic resonance angiography and digital subtraction angiography (DSA) are useful for diagnosing dural arteriovenous fistulas, and of these, DSA remains the “gold standard.” Most lateral foramen magnum dural arteriovenous fistulas need to be treated due to their aggressive symptoms, and transvenous embolization presents the best options. During treatment, it is critical to accurately place the microcatheter into the fistula point, and intraoperative integrated computed tomography and DSA data are very helpful. Other treatments, such as transarterial embolization, microsurgery or conservative treatment, can also be chosen. After appropriate treatment, most patients with lateral foramen magnum dural arteriovenous fistulas achieve satisfactory outcomes.