A 31-year-old female presented with a particularly rare hemangiopericytoma (HPC) in the right lateral ventricle manifesting as a 6-month history of visual disturbance and headache. Left hemianopsia and choked disc were identified by an ophthalmologist who referred her to us. Magnetic resonance imaging demonstrated a 5-cm homogeneously enhanced mass in the trigone of the right lateral ventricle. The tumor was totally removed by two stage surgery. The histological findings were consistent with HPC. HPC is very important to differentiate from meningioma and solitary fibrous tumors because HPC is more aggressive. The histological and immunochemical findings are important for the differential diagnosis. The present case showed no local recurrence or metastasis without radiation therapy for 4 years, indicating that radiation therapy is not absolutely imperative for patients with intraventricular HPC showing low MIB-1 staining index after total removal.