BACKGROUND Multimodal treatment approaches for children with tumors of the central nervous system (CNS) have markedly contributed to improved survival. Before 1970, the survival rate among children with medulloblastoma, the most common malignant CNS tumor in children, was about 20%. At present, in contrast, cure can be achieved in more than 75% of children with a favorable constellation of risk factors. In this review article for clinicians, we give an overview of the current understanding of the pathology, presenting manifestations, early diagnosis, and treatment of CNS tumors in children and adolescents. METHODS We report the research findings of the German Treatment Network "HIT" and selectively review the pertinent literature. RESULTS Treatment-optimizing clinical trials have improved survival from all types of CNS tumors in children and adolescents. Biological features of the tumors now serve as the basis for improved stratification for multimodal, risk-adapted treatment. Targeted biological strategies are being developed. Difficulties remain, however, in the care of infants with CNS tumors and in the treatment of metastatic disease, tumors of certain histological types, and tumors in certain anatomical sites. Many of the affected children suffer from late effects of their disease and its treatment that can irreversibly impair their development. CONCLUSIONS Children with a suspected or confirmed diagnosis of brain tumor should be referred early to a center with the relevant experience. Standardized diagnostic and therapeutic methods have markedly improved the chance of cure. Current research on molecular signaling pathways seems likely to lead to the development of new treatments, particularly for tumors currently associated with lower rates of survival. The long-term side effects of treatment must be systematically monitored so that they can be avoided in future, and so that appropriate support measures can be provided to the affected children.