Purpose. To describe the cause, diagnosis, and management of a case of bilateral corneal keloid. Methods. We describe a 17-year-old white boy with enlarging nontraumatic bilateral corneal scars whose growth was exacerbated by a superficial keratectomy. The patient underwent a penetrating keratoplasty (PK) in his left eye. Light and electron microscopy of the corneal button were performed. Results. The histopathologic and ultrastructural features of the corneal button were haphazardly arranged collagen fascicles with activated fibroblasts but no inflammatory cells. The clinical outcome was excellent, although there has been continuous growth of the outer margin of the initial lesion not included in the PK. This growth has not affected vision. The unoperated right corneal lesion progressively enlarged during these years. Conclusion. A corneal keloid, although unusual, should be suspected in cases of enlarging white glistening avascular corneal scars regardless of a traumatic antecedent. Light and electron microscopy confirmed the diagnosis. Management is by PK when the visual axis is involved and carries an excellent prognosis.