Tumors of the third ventricle in children raise specific problems, owing to their clinical presentation, pathology, treatment, and outcome. The clinical presentation is often unspecific at that age, many cases presenting with isolated macrocrania or delayed milestones. Some histological types are specific of the pediatric group, such as the pilocytic astrocytoma or hypothalamic hamartoma, and others types which are exceptional in adults are relatively common in children, such as choroid plexus tumors. Surgery is especially dangerous in young children, because of the blood loss, and the hemispheric collapse associated with the large hydrocephalus which is the rule in these patients. External irradiation is associated with a high rate of complications, above all a progressive intellectual delay, and endocrine disorders. Chemotherapy has reduced the indication of irradiation, and, in some cases, can be performed preoperatively to reduce tumor volume and intraoperative blood loss. The therapeutic approach to these patients is thus pluridisciplinar, and should be tailored for each case, with a follow-up protracted well into the adult age.