An 8-year-old Scottish terrier was presented with a red and painful right eye. Slitlamp biomicroscopy and ocular ultrasound demonstrated the presence of a large mass in the anterior chamber. Enucleation was performed one week after initial presentation. Histological examination of the eye revealed a relatively well-delineated mass distorting the dorsal iris and occupying most of the anterior chamber. The tumor was composed of fully differentiated adipose tissue and normal hematopoietic cells of the three major blood-forming elements. These findings were most consistent with the diagnosis of an anterior chamber myelolipoma distorting the iris. To the author's knowledge, this is the first report of an intraocular myelolipoma in any species.