Four patients who experienced the sudden onset of anterior uveitis with large keratic precipitates and dense vitreous opacities developed confluent yellow-white swellings and exudates in the peripheral retina and sheathing and obliteration of retinal arteries. After absorption of the exudates, atrophic patches in the peripheral retina and a funnel-shaped retinal detachment with many tears appeared. Angiograms showed retinal edema, fluorescein leakage from the choroid in the affected areas, and perivasculitis of the retinal arteries. Although treatment seemed unable to alter the course of the disease, in one case the retina reattachment after vitrectomy and filling of the vitreous space with silicone oil. Histopathologic studies disclosed extensive atrophy and degeneration of the outer retinal layers and pigment epithelium and occlusion of the retinal vessels. An infection may trigger the uveitis, leading to an autoimmune sensitization against rods and cones that causes severe local immune-complex disease and retinal vasculitis.