Corneal and limbal dermoids are uncommon choristomatous corneal tumors. They clinically present as round or oval, whitish or yellowish cones protruding on the anterior surface of the eyeball. They are composed of ectodermal (keratinized epithelium, hairs, sebaceous and sudoriferous glands, nerves, smooth muscles and, less frequently, teeth) and mesodermal elements (fibrous tissue, fat, blood vessels and cartilage) combined in different proportion. If fat dominates in histology of the tumor, it is called a lipodermoid. A case of a two-year old boy with a large corneal dermoid on the right eye is presented. Dermoid covered almost the whole cornea and was associated with adherence of the atrophic iris to the posterior corneal surface and partial congenital cataract. Surgical therapy included excision of dermoid together with the superficial lamellae of the corneal stroma and penetrating keratoplasty. Satisfactory tectonic and esthetic outcome was achieved and has been maintained for four postoperative years now.