Intraventricular schwannoma is extremely rare, with only 35 cases reported to date in the literature. Consequently, its etiology and pathogenesis are still unclear, and thus require further investigations. Here, we report on and discuss a rare case of intraventricular schwannoma to elucidate on this matter.A 26-year-old male patient was admitted to our institution with a one-month history of headaches and left hemianopsia. At diagnosis, magnetic resonance imaging of the brain revealed a well-demarcated mass with surrounding edema in the right lateral ventricle. Total resection of the tumor was performed by a trans-sulcal approach through the right parietal lobe. Upon surgery, it was observed that the tumor was attached to the choroid plexus without invading the wall of the right lateral ventricle. The respective histological examination confirmed the diagnosis of intraventricular schwannoma.Six months after the surgery, there was no recurrence. Additionally, during this follow-up period, the patient did not develop any neurological deficit, including visual field narrowing or parietal symptoms, such as acalculia and right-left, finger, and space agnosias.Although intraventricular schwannomas are rare, 35 cases have already been reported to date. We emphasize the importance of diagnosing such cases correctly to increase knowledge on the origin and pathogenesis of intraventricular tumors, which would facilitate disease management.