Sleep apnea in neurologically compromised children is common but underrecognized. It can be secondary to diseases at all locations on the neuroaxis and may independently alter their presentation, severity, and course. As a primary and secondary illness, it is associated with significant neurological morbidities. In its severe manifestation, it can cause life-threatening short- and long-term systemic morbidities. The authors review the most recent and relevant literature and provide the pediatric neurologist with a framework with which to identify children at risk.