Ocular malformation in association with ipsilateral facial nevus of Jadassohn

A 13-year-old boy had ocular malformations and a sebaceous nevus of Jadassohn. The salient features included left frontoparietal alopecia, a pigmented flat nevus anterior to the left tragus, and a left-sided fleshy corneoscleral limbal mass. Histopathologic analysis showed a complex choristoma of lacrimal tissue and cartilage, together with intrascleral cartilage and bone. Deformity of the left sphenoid bone, dilatation of the left lateral ventricle, and localized atrophy of the left cerebral hemisphere were evident, but the patient had no history of seizures, no neurologic deficit, and normal mentality. Patients with a sebaceous nevus of Jadassohn should be examined for ocular malformations and watched closely for the development of malignant nevoid tumors within the area of the nevus.