A 47-year-old woman presented with unilateral ventricular enlargement detected by magnetic resonance imaging during a medical checkup. Neuroendoscopic exploration identified a multilocular lesion in which dark red fluid formed a niveau near the right side of the foramen of Monro. The diagnosis was intraventricular cavernous angioma. Restricted flow of cerebrospinal fluid at the foramen of Monro was observed. Xanthochromia, which seemed to be due to previous bleeding, was observed at the fornix. When the neuroendoscope touched the angioma, the wall collapsed and bled. Endoscopic removal of the angioma was abandoned, and craniotomy and resection of the angioma were performed. No new neurological anomalies were observed after surgery. Preoperative diagnosis of intraventricular cavernous angioma is difficult based on neuroimaging. Neuroendoscopy is effective for diagnosis and the decision-making process regarding treatment.