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To report the MRI, myelographic, and angiographic findings as well as the clinical and radiologic time course of an intracranial dural arteriovenous fistula (DAVF) leading to cervical myelopathy; and to review the pertinent literature.Cervical myelopathy from an intracranial DAVF draining into spinal medullary veins is extremely uncommon. However, knowledge about the MR features of these lesions is important because an improper diagnosis might result in delayed or incorrect treatment.In a patient with progressive cervical myelopathy, T2- and proton density (PD)-weighted MRI, contrast-enhanced T1-weighted images, and a contrast-enhanced MR angiogram of the cervical spinal cord were acquired. Additionally, intraarterial digital substraction angiography (DSA) of the right and left common carotid arteries was performed.MRI findings included swelling of the cervical spinal cord, hyperintensity of the cervical cord on T2- and PD-weighted MRI, and an enlarged vessel at the ventral surface of the cord on MR angiography. No parenchymal contrast enhancement of the spinal cord was noted on T1-weighted MRI. DSA revealed an intracranial DAVF fed by four branches of the left external carotid artery and draining into spinal medullary veins. The fistula was treated with endovascular embolization, leading to considerable clinical improvement of the patient.To avoid an improper diagnosis or a delayed or incorrect treatment of myelopathy resulting from an intracranial DAVF, cerebral intraarterial angiography may be indicated in cases of otherwise unexplainable cervical myelopathy.