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Nevus sebaceus rarely occurs as part of a syndrome consisting of central nervous system and ophthalmologic abnormalities. We describe a case of nevus sebaceus associated with an epibulbar complex choristoma and colobomas of the optic disc and peripapillary choroid, and review the dermatologic, ophthalmologic, and neurologic literature on the nevus sebaceus syndrome. When associated with other developmental abnormalities, nevus sebaceus and epidermal nevus have erroneously been considered to be a single entity. In this article, we delineate and emphasize the ophthalmologic abnormalities associated with the nevus sebaceus syndrome.

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