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Choristomas inside the eye are rare and they commonly involve the uveal tissue and the optic nerve head mostly in the form of ectopic glandular tissue and choroidal osseous choristoma. Our case is unique in its intraocular retrolental location, composition of chondroid tissue and fat, in addition to the fact that it was found within a microphthalmic globe with other interesting histopathological findings. We report a case of an incidental finding of intraocular choristoma with associated microphthalmia, genetic testing may be useful for es