Central nervous system (CNS) tumors represent the most common solid tumors occurring in children with an age-standardized incidence rate of 5.61 and 7.26 per 100,000 persons during childhood and adolescence, respectively. CNS tumors are one of the leading causes of cancer related mortality in children as well as adolescents and young adults (AYAs).1 Pediatric CNS tumor survivors develop unique long-term sequelae including neurological deficits that impact their quality of life. 2, 3, 4, 5

Fortunately, in the last two decades we have witnessed several important advances in pediatric neuro-oncology leading to improved understanding of tumor biology as well as identification of biomarkers leading to more accurate diagnosis, prognosis, and risk stratification. This is reflected in the 2021World Health Organization's (WHO) Classification of Tumors of the CNS (CNS5) that integrate histologic and molecular features into a combined diagnosis. Coupled with the development of novel therapeutics, this has enabled personalization of treatment regimens to maximize survival while minimizing long term morbidities. 6

In this review, we will focus on an overview of the most frequently encountered primary pediatric CNS tumors (gliomas, ependymomas and medulloblastomas) along with the general approach to diagnosis and management.