To study the clinical characteristics of intraventricular epidermoids, we analysed retrospectively 12 patients treated in our hospital and reviewed the literature with regard to clinical manifestations, imaging features, diagnosis, surgical procedures and prognosis. Four patients with lateral ventricle epidermoid and eight in the fourth ventricle were included in this group. Intraventricular epidermoids are characteristically hypodense non-enhancing lesions on CT scans. MRI reveals them to have long T1 and T2 relaxation times with slight mass effect. Total removal is ideal, but special attention should be paid to preservation of important neurovascular structures. Close proximity of tumours to cranial nerves and the brain stem pose technical difficulties in total removal. Cranial nerve dysfunction and aseptic meningitis are the main postoperative complications. Long-term prognosis for patients with intraventricular epidermoids and well-preserved neurological conditions is good, even in the case of subtotal excision. Clinical follow-up and MRI allow earlier diagnosis of recurrence.