Massive retinal gliosis (MRG) is a rare, benign intraocular condition that may develop in association with long-standing eye conditions including chronic inflammation, vascular disorders, glaucoma, trauma, or congenital abnormalities. It is thought to represent a nonneoplastic reactive tissue response to retinal injury. Here, we describe an unusual case of bilateral MRG in association with retinopathy of prematurity. To our knowledge, this may be the first report of such an occurrence. The differential diagnosis of MRG is discussed with specific emphasis on its relationship to vasoproliferative tumor of the retina and presumed acquired retinal hemangiomas. In addition, we hypothesize that MRG, vasoproliferative tumor of the retina, and presumed acquired retinal hemangiomas may represent different phenotypes along a spectrum of the same disease process.