Management of adults with hydrocephalus treated during infancy or childhood represents a challenging process. Optimal neurosurgical management of adult patients with congenital hydrocephalus requires close attention to the complex issues of transitioning care, shunt management, and neuropsychological development. The intricacies of a patient's complicated medical history and the inherent differences between pediatric and adult neurosurgical practices contribute to the complex transition process. Many pediatric neurosurgeons routinely face the issue of transitioning patients with congenital hydrocephalus from pediatric to adult-oriented care. An adolescent's intellectual and behavioral development and their ability to function independently represent critical factors impacting their transition to adult care. Endoscopic third ventriculostomy (ETV) can be used to functionally convert appropriately selected hydrocephalic patients to shunt independence. Recurrent headaches and shunt malfunctions in the setting of slit ventricle syndrome (SVS) represent challenging problems for adults with congenital hydrocephalus and their care providers.