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 sudorifero
us glands, nerves, smooth muscles and, less frequently, teeth) and mes
odermal elements (fibrous tissue, fat, blood vessels and cartilage) co
mbined in different proportion. Lf fat dominates in histology of the t
umor, 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 a
lmost the whole cornea and was associated with adherence of the atroph
ic iris to the posterior corneal surface and partial congenital catara
ct, Surgical therapy included excision of dermoid together with the su
perficial lamellae of the corneal stroma and penetrating keratoplasty.
Satisfactory tectonic and esthetic outcome was achieved and has been
maintained for four postoperative years now.