Choristomas have been reported in the orbit, cornea, conjunctiva, uvea, retina, and optic nerve. Many reports have described the choristoma mainly in the epibulbar area, but little is known of the choristoma that occurs intraocularly. We reviewed the literature and summarized the reports that described the choristoma inside the eyes. Iris, ciliary body, choroid, and optic nerve head are the commonly affected tissues. In the anterior uveal tissue, an ectopic lacrimal gland is common and appears as a pinkish, nodular, fleshy mass. Its clinical course largely depends on the enlargement of the accompanying cyst. Osseous choristoma is mainly found in the choroid in young female patients. Although it is regarded as a benign tumor, it grows progressively and can decrease visual function. Treatment consists of close observation and possibly surgical intervention. Fortunately, most of the intraocular choristomas do not need aggressive treatment.