A male child was born with bilateral corneal opacification. His maternal uncle and a male first cousin of the patient's mother also had been born with identical bilateral corneal opacification that was pathologically proven to result from corneal dermoids. Histopathological examination of tissue removed from the present patient at the time of lamellar keratoplasty revealed thickened, keratinized epithelium, absent Bowman's membrane, and dense, irregular bands of highly vascularized collagenous connective tissue. No adnexal appendages were identified. The remainder of the ocular and general physical examination results was normal. In view of these clinical and histopathological similarities, the diagnosis of corneal dermis-like choristomas was made. To our knowledge, this report is the first to describe a family with X chromosome-linked recessive transmission of this condition.