A 5-year-old girl was referred for a growing tumor of the left eye. Visual acuity was 20/20 OU. Biomicroscopy showed a nonpigmented vascular mass of the inferior ciliary body with invasion of the iridocorneal angle and iris. The tumor was immediately suggestive of medulloepithelioma owing to the presence of superficial cysts (Figure 1). Staging, including magnetic resonance imaging and high-frequency ultrasonography, showed a tumor extending from the 5-o’clock position to the 7-o’clock position with invasion of the pars plana but sparing the ora serrata. The tumor had doubled in size by the time of staging. An iridocyclectomy was performed, followed by irradiation depending on the results of histological examination. Macroscopically, the ciliary body mass was a well-circumscribed, grayish tumor measuring 10 × 5 mm. Microscopically, this tumor corresponded to a proliferation of immature neuroepithelial cells forming cords and tubules in a loose stroma and displaying cystic spaces, necrotic foci, and glial tissue. The tumor cells exhibited nuclear pleomorphism, and mitotic activity was brisk (10 mitoses per high-power field). There were retinoblastoma-like areas with Flexner-Wintersteiner rosettes. There was no microscopic infiltration of the tumor's surgical margins. On immunostaining, the neoplastic cells expressed retinoblastoma protein, vimentin, and neural markers such as CD10, CD56, and synaptophysin. They were negative for cytokeratin and S-100 protein. On ultrastructural examination, zonula adherens were found between adjacent neoplastic cells and were localized near the lumen of tubules. Cilia were observed inside the lumens. Microtubules were present in the cytoplasm of the tumor cells (Figure 2).