Medulloepithelioma Masquerading as Chronic Granulomatous Uveitis

To report a case of ciliary body medulloepithelioma presenting as granulomatous uveitis. Patient and A 3-year-old girl presented with chronic granulomatous anterior uveitis in the right eye following unilateral cataract extraction and IOL implantation which was unresponsive to topical steroids and intra-vitreal injection of vancomycin and triamcinolone acetonide. One month later, white masses appeared over the iris surface. Histopathologic examination of the iris biopsy disclosed ciliary body medulloepithelioma. The patient was referred for plaque therapy due to the aggressive behavior of the tumor and finally, the eye was enucleated due to getting blind and painful and high suspicious of malignant behavior of the tumor. Medulloepithelioma is a rare congenital ciliary body tumor that presents with decreased vision, pain, leukocoria, and observation of a mass in the region of the ciliary body, anterior chamber and iris. Secondary glaucoma, cataract, uveitis, rubeosis iridis, retinal detachment, and ectopia lentis may develop later. The tumor may be locally aggressive and rarely metastasize. If extraocular extension does not occur, iridocyclectomy with tumor excision (in small tumors) and enucleation (in large tumors) appears to be the treatment of choice.