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Dural arteriovenous fistulas (DAVF) involving the craniocervical junction are uncommon lesions that may result in neurological deficits referable to posterior fossa structures and/or the spinal cord. We report on two patients with craniocervical junction DAVF whose venous drainage involved the cervical spinal cord. Both cases presented with progressive quadriparesis and parenchymal magnetic resonance signal abnormality of the cervical spinal cord. Both patients improved following embolization of the fistulas. AVF of the craniocervical junction are an uncommon, but important cause of treatable neurological deficits referable to this region of the nervous system.