Bilateral total corneal and conjunctival choristomas associated with epidermal nevus

A 4-year-old boy had been noted from birth to have bilateral masses obscuring the entire outer portion of the eyes. Systemic findings included mild growth retardation, multiple cortical lesions of the distal metaphyseal ends of the lower extremities, and diffuse skin pigmentation of the right side of the body. The epibulbar lesions were excised; on the right side, there was a thin cornea that necessitated corneal transplantation. The diagnosis of composite epibulbar choristomas associated with linear epidermal nevus was established by pathologic examination. This case report as well as a literature review establish the association of epibulbar choristomas with cutaneous linear nevi of the epidermal and sebaceous types.