A malignant and uncommon cancer of the central nervous system arising from the medullary epithelium. Usually develops in the parietal and temporal lobes, but may occur in the eye (intraocular medulloepithelioma). It typically affects children and infants. May be present at birth, but usually not diagnosed until early childhood. Characterized by headache (increased intracranial pressure), rubeosis iridis, leukocoria, focal neurologic deficit, and visual impairment. Signs and symptoms include pain, an ocular mass, glaucoma, cataracts, and seizures.

Prognosis may be good if treated before tumor spreads, but may not spare the eye.

Treatment usually involves enucleation. Other treatments may include tumor resection, radiation therapy, and chemotherapy.