Duchenne muscular dystrophy (DMD) is an X-linked recessive genetic disorder whose ophthalmic associations most commonly consist of pigmentary fundus changes and scotopic electroretinogram abnormalities. A 23-year-old man with advanced DMD and associated cardiopulmonary compromise complaining of floaters presented with a striking retinal vasculopathy characterized by vitreous hemorrhage, neovascularization, capillary drop-out, and prominent saccular venular aneurysms diffusely throughout the fundus of both eyes. This vasculopathy was promptly treated with panretinal phocoagulation with marked improvement to at least 1 year. This report suggests that early identification and prompt treatment of DMD may be important in managing this condition.