This report concerns a 3-month-old boy where neuroimaging examination showed a large, well-circumscribed, mildly heterogeneous tumor arising in the left ventricle. Pathological findings were compatible with a medulloepithelioma. A survey of published cases of medulloepitheliomas showed this tumor to be highly malignant, possibly displaying the entire range of differentiation from embryonal primitive neuroepithelium to mature cells and usually involving the cerebral hemispheres with a very poor prognosis in this location. On the other hand, medulloepitheliomas occurring in the eye or the orbit generally benefit from a gross-total resection and may present a good prognosis. Curiously, the patient reported here is doing well 7 years after the resection without any postoperative treatment. The exclusive intraventricular location of the tumor and its gross-total resection clearly seems to have contributed to this unusual recovery.