Medulloepithelioma is a rare tumor affecting children and adolescents. It usually grows in unpigmented ciliary body but cases in which it originates from the retina or the optical nerve have been reported. The tumor usually appears in the first decade of life but it can also affect adults, in which case it is almost always unilateral tumor. The tumor progression is slow and it usually does not metastize. Treatment involves enucleation of the affected eye and the prognosis is usually favorable. This case study describes an 18-year-old patient with extremely rapid progression of a non-teratoid meduloepiteliom, containing histologic structures resembling retinoblastoma. It is an uncommon and interesting case on several levels: anamnesis, rapid progression, and histologic findings. Based on this case, it is apparent that malignant meduloepitheliom may clinically manifest itself even after the fifteenth year of life, it may grow agressively, and it may metastize into regional lymph nodes. However, enucleation treatment coupled with chemotherapy may still be successful.