Abstract
Chiari II malformation is commonly associated with hydrocephalus requiring early cerebrospinal fluid (CSF) diversion and may coexist with complex supratentorial developmental abnormalities. As a result, patients with severe congenital structural brain anomalies and long-term ventriculoperitoneal (VP) shunting often experience neurodevelopmental impairment and are at increased risk of epilepsy. We report a 17-year-old girl with Chiari II malformation, repaired myelomeningocele, and ventriculoperitoneal shunting since birth (revision at five years of age), demonstrating marked ventricular improvement over a 16-year interval and a prolonged seizure-free interval despite severe congenital supratentorial malformations.