ABSTRACT
Rosette-forming glioneuronal tumor (RGNT) is a rare and indolent mixed glioneuronal tumor involving primarily the fourth ventricular region and occurring predominantly in young adults. We present a case of a 44-year-old woman presented with progressive headaches, vomiting, and a sudden decreasing level of consciousness. The magnetic resonance imaging showed a regular lesion within the anterior portion of the third ventricle and the patient underwent an endoscopic approach to remove the tumor that was exclusively within the anterior portion of the third ventricle. Histopathology showed an RGNT that was totally removed. We also report some unusual complications that are described in the literature and are related to ventricular endoscopy such as seizures and hydroelectrolyte disorders. With two years of follow-up, the patient had no complaints and no tumor progression was observed.