Treatment of infants and very young children with tumors of the CNS is challenging due to the vulnerability of the developing nervous system. Historically, treatment has relied upon surgery and radiation for control of malignant tumors with limited success and severe long-term consequences in most young patients. More recent studies have attempted to utilize chemotherapy and second-look surgery in attempts to reduce, delay or avoid radiation. Identification of prognostic factors for specific tumor types may distinguish which patients should receive more aggressive therapies and which patients can potentially avoid them and their potential side effects. Achieving successful tumor control while optimizing quality of life for survivors is the major goal of recent research.