Nine patients had birdshot retinochoroidopathy. Associated ophthalmologic findings included rhegmatogenous retinal detachment, rubeosis iridis, glaucoma, and a high incidence of disciform macular degeneration. Four patterns of birdshot spot distribution were noted on fundus examination. Immunologic studies showed a significantly elevated percentage of EA rosettes and an elevated C4 complement level. Electroretinograms showed b-wave amplitude reduction, with a disproportionate implicit time prolongation. The etiology of this syndrome remains unknown.