Primary central nervous system (CNS) neoplasms are the most common solid tumors in children. During the last two decades progress in diagnostic procedures and treatment has been made. New techniques of imaging (CT, MRI), neurosurgery, intensive care and the introduction of intensive chemotherapy and refined radiotherapy had the greatest impact on this progress. Nevertheless, out of all pediatric malignancies this group of patients has the worst prognosis. To improve survival optimal treatment modalities are searched for. This paper presents brain tumors epidemiology, etiology aspects, classification, clinical symptoms, diagnostic procedures and current treatment strategies in CNS tumors in children such as: medulloblastoma/PNET, high and low grade gliomas, ependymomas, germ cell tumors and brain stem tumors. Age of the patients is considered in all tumors as there is a specific philosophy of treatment in small children. We are also presenting directions of recent clinical trials and the aims of pediatric oncologists in improving the effectiveness of treatment and quality of life of a cured child.