Purpose: To report a case of intraocular malignant teratoid medulloepithelioma, an embryonal tumor with extremely rare presentation in adults.
Method: The case of a 36-year-old male with intraocular malignant teratoid medulloepithelioma, primarily diagnosed as intraocular choroidal melanoma 6 year back, is described and the literature on this subsect is reviewed.
Result: The patient presented with blurring of vision in right eye on both distance and near vision of two-month duration. In association to this he has also a history of itching and pain of the same duration. Six years back he was diagnosed as intra ocular choroidal melanoma with histopathological examination.

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