The clinical course and pathological findings in an infant with a congenital corneal opacity, aniridia, and cataract of the right eye and anophthalmia of the left orbit are reviewed. Although the lesion was thought to represent a dermoid pre-operatively, the keratoplasty specimen revealed disorganized and vascularized connective tissue, with thickened, keratinized epithelium. Bowman’s and Descemet’s membranes as well as the endothelium were absent. These histopathologic findings were felt to represent a congenital corneal keloid. The co-existence of aniridia in the involved eye and anophthalmia of the contralateral socket are hypothesized to be evidence that a primary ocular developmental disorder, rather than a reparative process, resulted in the corneal alteration. The difficulties of penetrating keratoplasty in lesions of this sort are examined.