A 14-year-old gray gelding was presented for investigation of a visible, pale-colored ocular mass in the right eye. An intraocular mass was identified clinically and ultrasonographically as originating from the superior nasal quadrant of the ciliary body and retina. The mass occupied the majority of the vitreous chamber and some of the superior anterior chamber of the eye. The affected eye was blind. Following exenteration, a primary intraocular primitive neuroectodermal tumor (i.e. a retinoblastoma/medulloepithelioma), a rarely described intraocular mass in adult horses, was identified by pathologic examination. The gelding returned to normal use following a short recovery period.