Abstract
A 29-year-old woman experienced long-term phthisis bulbi owing to previous refractory idiopathic posterior uveitis since childhood and eventually underwent evisceration. Histological examination revealed that the retina was intermingled with the unusual glial mass lesion, which contained spindle cells with the elongated fibrillary process and columnar cells surrounding the round lumen with pseudorosettes formation. Mitoses were rarely seen. The tumor cells expressed S100, glial fibrillary acidic protein, CD99, and vimentin, while Ki67 was not detected. The final diagnosis was intraocular retinal ependymoma. Intraocular ependymoma was extremely rare and lacked comprehensive literature reviews. Our case is the very first example of retinal ependymoma in East Asia. Evisceration or enucleation followed by regular scrutinization is regarded sufficient for treating this condition.