Pediatric III ventricular tumors constitute an heterogeneous group of neoplasms which share some specific characteristic from the surgical point of view. All these tumors are rare and tend to remain clinically silent until they produce hydrocephalus. By excepting colloid cyst, which may induce an abrupt deterioration in clinical condition because of their critical location in the CSF pathways, most of these tumors reach a considerable large size before being recognized. Macrocrania in infants, false localizing signs, namely sixth cranial nerve palsy, and, more rarely, hemiparesis secondary to transtentorial herniation are the most common clinical manifestations of the associated chronic hydrocephalus. Although the ventricular dilation is often a favorable feature for the surgical removal, the unfolding of the thinned cerebral cortex and the consequent hemispheric subdural fluid collections represent the most typical surgical complication of the surgical treatment often requiring additional surgical procedures. The most typical tumors are the choroid plexus and the colloid cysts, the first of them relatively common, the second exceedingly rare in the pediatric population. Subependymal giant cell astrocytomas and subependymal are also quite known intraventricular tumors arising from the subependymal glia. Finally, midline gliomas from the adjacent parenchima and craniopharyngiomas from the suprachiasmatic cistern may rarely invade the ventricular system. Finally in children, meningiomas and teratomas may expand within the III ventricle, often without any recognizable origin from the ventricular wall. Choroids plexus papillomas represent only 0.4 to 0.6% of intracranial tumors, but up to 90% to these tumors occur within the first 2 years of life. The young age, the commonly large size and hypervascularity of these tumors may require tailored surgical strategies, including staged surgical excision, preoperative embolization or chemotherapy. The control of impaired CSF dynamics still remains one difficult to solve postoperative complication. Bilateral forms carry the worst prognosis in terms of postoperative hydrocephalus and neurodevelopmental outcome. Colloid cysts are extremely rare in children. The endoscopic excision is nowadays preferred in the young patients due to the large experience generally available with the endoscopic technique in the pediatric department. Subpendymal giant cell astrocytomas, the typical neoplasms of tuberous sclerosis is characterized by an unpredictable clinical evolution. In growing tumors causing obstructive hydrocephalus surgical excision is regarded as the most reliable surgical option due to the multiple failures of bilateral CSF shunting therapy observed in the subjects undergoing the extrathecal CSF shunt placement. In summary, most of the III ventricle tumors in children are benign in nature. Consequently, the surgical treatment remains the most effective therapeutic options. However, the deep location of these tumors, the strict anatomical relationships with important functional structures, the frequently associated hydrocephalus, their large size together with their relatively rare occurrence which may prevent reaching a sufficient experience, suggest that these tumors should be dealt with in specialized pediatric neurosurgical centres.