Malignant brain tumours (mainly medulloblastoma, glioma, and ependymoma) are the first cause of solid tumours in children, and a major cause of mortality from cancer in paediatrics. The most frequent circumstance of discovery is intracranial hypertension. Early and atypical signs should give the alarm and call for emergency neuroradiological explorations. Major therapeutic advances have been made in the last ten years in the fields of neurosurgery, radiotherapy and chemotherapy of brain tumours in children. Multidisciplinary care is now indispensable usually as part of multicentre cooperative clinical trials. Therapeutic approaches are guided by a concern not only for effectiveness, but also for low toxicity, in order to reduce the long-term sequelae often caused by irradiation of the developing central nervous system.