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Malignant teratoid medulloepithelioma is an uncommon unilateral intraocular tumor, occuring typically in children. This report concerns a 4-year-old boy, who presented with loss of vision, pain and proptosis of the left eye and showed mass in between eyelids. Histopathologically, the tumor was composed of pseudostratified primitive- appearing epithelium dispersed in cords, strands, tubules & glands, which were separated by a cystic spaces, filled with pale eosinophilic material. Foci of glial tissue, cartilage , bone, skeletal muscle and fatty tissue were recognized. In addition, scleral extension of tumor, Homer-Wright like and Flexner-Wintersteiner like rosettes and foci of necrosis were also present. The differentiation from retinoblastoma was discussed. Key Words: Malignant medulloepithelioma, eyeball, heteroplasia.