Intraocular paraganglioma is a very rare tumor. There have only been 4 reports of this tumor location in the literature, which arose in the choroid and iris. We present a patient with an intraocular paraganglioma masquerading as an ocular melanoma, clinically and radiographically, that required careful histological and immunohistochemical analysis for differentiation. Due to the sparse reports, we feel documentation of this patient, as well as a review of previous presentations, is necessary to contemplate clinicopathological correlations.
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