This is a report of two cases of optociliary shunt vessels in patients with neonatal hydrocephalus. Complete disappearance of the optociliary shunt vessels occurred after surgical procedures to normalize intracranial pressure. The clinicopathological significance, prognosis, and treatment of patients with acquired optociliary shunt vessels is discussed. This is also the first report of the association of optociliary shunt vessels and neonatal hydrocephalus.