Introduction: Papillary Glioneuronal Tumor (PGNT) is a grade I tumor based on the new World Health Organization CNS tumor classification. Its special feature concerns its biphasic histologic pattern of both glial and neuronal elements. Because of the rarity of this entity, literature is mostly based on case reports. Research Question: The objective of this paper is to display our experience treating endoscopically a papillary glioneuronal tumor located into the third ventricle in an adult patient and to highlight the main points of the literature. Material & Methods: A 75-year-old patient with known Parkinson disease presented with episodes of loss of consciousness and gait disorders in our institution. Brain MRI demonstrated enlargement of ventricular system due to an obstruction caused by an intraventricular tumor in the third ventricle. Results: Patient underwent an endoscopic subtotal removal of the tumor. Postoperative brain CT scan revealed minor residual of the tumor and patient was discharged 2 days after surgery in good clinical condition. Histopathological analysis of the tumor samples matched the diagnosis of PGNT. Postoperative follow-up in 1 month demonstrated great neurological improvement. Discussion & Conclusion: Literature reports that total surgical resection is the treatment of choice in almost all cases of PGNT giving the most favorable clinical outcome. Only 4 cases of PGNT located in 3rd ventricle have been recorded and data for endoscopic management found only for one of the cases concerning a pediatric patient. Therefore, we report for first time removal of third ventricle papillary glioneuronal tumor in adult patients via the endoscopic technique.