A premature infant who had vascularized only Zone I developed bilateral progressive hemorrhaging in the posterior poles. Subsequently, vitreous hemorrhages and retinal detachments occurred without any progression through Stages I, II, or III of retinopathy of prematurity. This clinical presentation has not been reported previously. These findings suggest profound underlying abnormalities of funduscopically normal Zone I retinal vasculature. Pathologic findings were consistent with end-stage retinopathy of prematurity.