BACKGROUND: Few cases of corneal perforation in a premature infant have been described in the literature. METHODS: Identical twins were born at 30-weeks gestation. One twin developed a corneal epithelial defect, infiltration, and perforation with extrusion of intraocular contents, requiring an emergency penetrating keratoplasty. The second twin developed a progressive corneal opacity, requiring a lamellar keratectomy. RESULTS: After penetrating keratoplasty, the patient developed absolute glaucoma with buphthalmos, leading to enucleation. The twin's lamellar keratectomy specimen proved to be a dermoid. CONCLUSIONS: One must be aware of the danger of the development of spontaneous corneal perforation with extrusion of intraocular contents in premature infants presenting at birth with an epithelial defect.