• A malignant teratoid medulloepithelioma (ME) first became symptomatic in a 41-year-old woman. Her eye was enucleated for advanced glaucoma when the patient was aged 43 years, making her the oldest patient known to have had this tumor. The tumor showed little epitheliomatous differentiation, but a large rhabdomyoblastic component and nodules of cartilage. It had extended over the retina, creating a change in macular reflex as an early clinical sign, and eventually produced large retinal contraction folds. The lens was invaded by tumor, and an active phakoanaphylactic uveitis was in progress. Three of the four MEs recognized in adults have been malignant.