Four patients, two men and two women ranging in age from 40 to 70 years, had birdshot retinochoroidopathy. The special features of this entity are diffuse posterior choroidopathy with associated vitritis and retinal vasculitis. Decreased central vision is caused by cystoid macular edema. In the early course of this disease, the cystoid macular edema may be responsive to corticosteroids. The diffuse choroidopathy is associated with abnormal retinal pigment epithelium and sensory retinal function on electrophysiologic testing. No previously described choroidal or retinal pigment epithelium diseases appear to resemble this entity.