The authors describe an extremely rare case of foramen magnum dural arteriovenous fistula (DAVF), Cognard type V, presented with medullary hemorrhage caused by venous varix on the lateral medullary draining vein embedded into the medulla oblongata. Following mild myelopathy for 3 days, a 20-year-old male developed dyspnea, generalized seizures, loss of consciousness, and finally cardiac arrest. After successful resuscitation, computed tomography scan (CT) of the brain was obtained and showed acute medullary hemorrhage. Subsequent magnetic resonance imaging of the brain revealed diffuse venous congestion or edema of the medulla with multiple dilated flow voids surrounding the medulla, more prominent on the left side, with venous varix embedded into the left-sided of the lower medulla. He was sent to the emergency department of the local hospital and intubated promptly. A few minutes later, the patient had a cardiac arrest. Digital subtraction angiography (DSA) demonstrated DAVF of the foramen magnum supplied mainly by dural branches of bilateral hypertrophic posterior inferior cerebellar arteries (PICAs), slightly by the posterior meningeal branch of the left vertebral artery, and the jugular branch of the left ascending pharyngeal artery (APA) originating from the occipital artery. Transarterial embolization through the bilateral dural branches of the PICAs was successfully performed using N-butyl-2-cyanoacrylate (NBCA), resulting in complete obliteration. The patient had excellence recovery and lost to annual follow-up. Seven years later, he had a recurrent of the fistula presented with occipital headache. DSA with angiographic CT in three-dimensional reconstruction and maximum intensity projection reformatted images clearly demonstrated the exact location of the DAVFs at the posterior rim of the foramen magnum, mainly recruited by the hypertrophic jugular branch of the APA originating from the occipital artery. The fistula was successfully treated surgically following transarterial embolization through the jugular branch of the APA using NBCA. Follow-up DSA confirmed complete obliteration of the DAVF. The patient has remained clinically asymptomatic 2 years after the operation.