Tumors of the pineal region account for 3 to 8% of the tumors involving the central nervous system. The most common are germ cell tumors (39%). Less common examples include teratomas, primitive neuroectodermic tumors, astrocytomas, and choriocarcinomas. Clinical presentation in pediatric patients is in direct relation to the anatomical structures surrounding the pineal region and to the associated hydrocephalus that is present in almost 90% of cases. The diagnosis of a tumor in the pineal region is based on radiological findings and the presence of tumor markers such as α-fetoprotein and human chorionic gonadotrophic hormone subfraction β. Neuroendoscopy is considered to be one of the minimally invasive techniques useful for the management of such patients. This minimally invasive approach to pineal region tumors allows the treatment of hydrocephalus in a safe and effective way, avoiding the complications derived from other procedures such as external ventricular drainage or shunt surgery.