Pediatric central nervous system (CNS) tumors are the most common solid tumors in children and comprise 15% to 20% of all malignancies in children. Presentation, symptoms, and signs depend on tumor location and age of the patient at the time of diagnosis. This article summarizes the common childhood CNS tumors, presentations, classification, and recent updates in treatment approaches due to the increased understanding of the molecular pathogenesis of pediatric brain tumors.