Atypical oculo-orbital complex choristoma in organoid nevus syndrome.

We report organoid nevus with left oculo-orbital choristoma fixing the globe in hypotropia, precanthal skin tag, and upper eyelid coloboma and right limbal dermoid. Computed tomography revealed a dilated lateral ventricle and cerebral atrophy. No neurologic deficit existed. The coloboma was repaired, choristoma debulked, and precanthal tag excised.