The authors report an unusual case of engorged epidural veins causing progressive cervical myelopathy after long-term cerebrospinal fluid (CSF) shunt therapy and intracranial hypotension. An 18-year-old woman, who had previously undergone shunt placement with a distal slit valve for a porencephalic cyst when 2 years of age, presented with progressive spastic quadriparesis, numbness, and gait difficulty. Postural headaches were absent and a lumbar puncture revealed low CSF pressure. Neuroimaging disclosed markedly engorged anterior epidural veins causing compression of the cervical spinal cord. The slit-valve shunt system was surgically removed and an external drain was placed. The patient's CSF pressure was gradually raised to clinically tolerable levels. Once the optimal pressure was identified, a programmable shunt was placed with the valve set at the same level. The patient's neurological status improved, and the epidural veins had returned to their normal size on follow-up imaging. The authors describe the unique treatment strategy used in this patient and review the literature on epidural venous engorgement as it relates to intracranial hypotension.