Symptomatic ventricular coaptation, or the slit ventricle syndrome, is frequently described and recognized as a clinical entity in the pediatric population. It is characterized by symptoms of shunt failure (i.e., ataxia, obtundation, nausea, vomiting, lethargy, irritability, and complaints of headache) and the CT findings of ventricular coaptation (slit-like ventricles). This study of twenty-two children with this syndrome reflects the variety of possible clinical presentations, and the variety of available treatment modalities. Multiple therapeutic approaches were required in seven of the patients. This illustrates not only an evolving treatment regimen, but also that a single treatment modality is not universally effective. Six patients needed only occasional symptomatic support. Blockage and/or removal of the shunt system was the definitive therapy in six patients, pressure augmentation in nine patients, and subtemporal craniectomy in one. This article outlines the theoretical pathophysiology, and a scheme for the management of patients with this syndrome.