Background: Intraocular medulloepithelioma arises from the primitive medullary epithelium and is diagnosed at a median age of five years. This tumor most commonly appears as a white, gray, or yellow-colored ciliary body tumor. The growth of medulloepithelioma is slow and it is locally invasive. Poor vision and pain are the most common presenting symptoms. The most common clinical signs include cyst or mass in iris, anterior chamber or ciliary body, glaucoma, and cataract. Case: A 22-month-old Caucasian female twin presented with leukocoria and poor vision in OS. Examination revealed normal findings OD and a mass in OS. Based on the clinical features of leukocoria, lens changes and a white cystic ciliary body mass in a young child, ultrasonographic, and transillumination features, the lesion was diagnosed as a non-pigmented ciliary epithelial medulloepithelioma. After enucleation, the diagnosis of malignant teratoid medulloepithelioma of the non-pigmented ciliary epithelium was confirmed. There was no evidence of tumor recurrence or systemic metastasis at three years follow-up. Conclusion: Medulloepithelioma in a child can present as a clinical triad of leukocoria, lens changes, and a white cystic ciliary body mass.

Background: Intraocular medulloepithelioma arises from the primitive medullary epithelium and is diagnosed at a median age of five years. This tumor most commonly appears as a white, gray, or yellow-colored ciliary body tumor. The growth of medulloepithelioma is slow and it is locally invasive....