Autosomal dominant vitreoretinochoroidopathy (ADVIRC), a recently described disease, is clinically characterized by a slowly progressive or stationary circumferential peripheral pigmentary retinopathy with fibrillar condensation of the vitreous. Histopathologic study of an 88-year-old patient with this disease showed disorganization of the peripheral retina with focally atrophic retinal pigment epithelium (RPE). Altered pigment epithelial cells surrounded retinal blood vessels and lined the internal limiting membrane. At the equator, a remarkable and possibly unique multifocal loss of photoreceptor cells was seen. An extensive preretinal membrane, consisting of condensed vitreous with cellular debris and layers of Müller cells, was demonstrated by electron microscopic examination and immunohistochemistry. Histologically, this entity has some similarities to and some differences from retinitis pigmentosa. The clinical features are distinctive.