Intraocular medulloepithelioma is a rare embryonal neuroepithelial tumor that originates from the undifferentiated medullary epithelium over the ciliary body or inner layer of the optic cup (Zimmerman 1971; Fuchs 1908). It commonly arises from the nonpigmented ciliary epithelium of the pars plicata and rarely in the iris, retina, optic nerve, and the central nervous system (Verhoeff 1904; Takei et al. 2007; Morris and Garner 1975; Vadmal et al. 1996; Green et al. 1974; Chavez et al. 2004; Sundaram et al. 2003; Steinkuller and Font 1997).