Extramedullary plasmacytoma of the iris and ciliary body is extremely rare. We present a case which was misdiagnosed as granulomatous uveitis with neovascular glaucoma, and underwent a trabeculectomy with mitomycin-c along with iris biopsy. The post-operative period showed early bleb failure and catastrophic growth of the suspected mass. Histopathological examination revealed a diagnosis iris plasmacytoma. Subsequent ultrasound biomicroscopy showed involvement of the iris and ciliary body. A prompt systemic workup was done, and an associated systemic plasma cell dyscrasia was ruled out. The affected eye was enucleated, and the patient remains disease free at the end of 3-year follow-up.
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