To report the postmortem histopathologic features that closely resemble the clinical features of parafoveal telangiectasis. Light and electron microscopy. Histopathologic features included macular edema; telangiectatic vessels; retinal, subretinal, and superficial retinal neovascularization; retinal pigment epithelial hyperplasia around neovascular aggregates; retinal-choroidal vascular anastomosis; and superficial pigmented cells with lipofuscin. The postmortem histopathologic findings in a 36-year-old woman with Down syndrome and other systemic conditions correlate with features noted in previous reports of presumed parafoveal telangiectasis.