The authors describe a patient with lower cervical dural arteriovenous fistula (DAVF), subsequently developing brainstem dysfunction following progressive myelopathy caused by extension of venous congestion from the cervicothoracic cord to the medulla oblongata. A 34-year-old woman suffered from progressive paraparesis with bowel/bladder dysfunction for 2 months. Before transferring to our institute, she obtained magnetic resonance imaging (MRI) of the neck, showing abnormal hyperintensity on T2-weighted sequence extending from the level of C1 to T5 with abnormal intradural flow voids along anterior more than posterior pial surfaces of the cervical spinal cord. Due to clinical deterioration, the patient was admitted before the schedule and obtained follow-up MRI, revealing progression of abnormal hyperintensity extending from the level of T7 to the lower medulla, representing progressive venous congestion. Contrast-enhanced magnetic resonance angiography (MRA) demonstrated a cervical DAVF at the C8 nerve root, supplied by a feeder originating from the deep cervical artery, and prominently engorged draining veins extending rostrally to the pial venous network around the medulla, and caudally to the thoracic cord. There was a venous pouch located in the right C7-T1 intervertebral foramen. During waiting for endovascular treatment, the patient developed respiratory dysfunction, needed endotracheal intubation. Emergency embolization was successfully performed with N-butyl-2-cyanoacrylate. MRI and angiography of the cervical spine, obtained few months after embolization, confirmed complete obliteration of the fistula and resolution of venous congestion. Three years follow-up MRI and MRA showed no recurrence of the fistula. The patient still had spastic paraparesis. Bowel and bladder dysfunction had gradually improved. Natural history of spinal DAVFs in lower cervical spine probably has more aggressive clinical course than thoracolumbar region. Prompt treatment should be performed before clinical deterioration due to brainstem dysfunction. Delayed treatment resulted in irreversible neurological deficit.