Schwann cell tumors arising within the neuraxis and in an intraventricular location are an exceedingly rare tumor entity of the brain. The authors present the first case of a cellular intraventricular schwannoma occurring in the fourth ventricle. The pertinent literature is reviewed. A 71-year-old female was admitted to the hospital with an incidental finding of a ventricular tumor. Computed tomography scanning and magnetic resonance imaging revealed a solitary contrast enhancing exophytic mass lesion within the fourth ventricle. Microsurgical excision via a midline suboccipital craniotomy and tonsillo-nodular approach led to complete tumor removal. Subsequent histopathological examination confirmed the diagnosis of an unsuspected primary intraventricular cellular schwannoma. A unique case of an initially unexpected benign schwannoma arising from the fourth ventricle that could be successfully treated by microsurgery and finally confirmed by histopathological analysis with excellent patient outcome is presented. Although highly uncommon, Schwann cell tumors of both benign and malignant nature may present as ventricular lesions and should be included as a differential diagnosis in patients with either solely intraventricular masses or intra- and extraaxial tumors with extension to the ventricles.