Central nervous system tumours are the most common solid tumours and second most common malignancy in pediatric age group. They are the leading cause of cancer related morbidity and mortality. It accounts for 3.5% of all deaths in the 1–14 years age group. Childhood central nervous system (CNS) tumors differ significantly from adult brain tumors in reference to their sites of origin, clinical presentation, tendency to disseminate early, histological features and their biological behaviour. Supratentorial tumors are more common in infants and children up to 3 years of age and again after age 10, whereas between ages 4 and 10 infratentorial tumors are more common. The initial workup of patients with brain tumors must include a complete history, physical examination, imaging and biopsy confirmation of primary. The management of pediatric brain tumours is important due to their high incidence, challenging aspects of surgery and high mortality. Many CNS malignancies, which were once universally fatal are now curable with multimodality approaches that integrate surgery, chemotherapy and radiotherapy. In this chapter, we will discuss these issues in detail and summarize the ongoing efforts to reduce the morbidity and mortality in pediatric CNS tumours