Birdshot retinochoroidopathy is a rare, chronic, bilateral, posterior uveitis with a distinctive clinical presentation and a strong genetic association. Middle aged white people of northern European extraction are most commonly. The pathogenesis is unknown, but HLA-A29 positivity appears to confer predisposition and retinal autoimmunity seems to play a role. Clinically, there are patches of postequatorial choroidal and retinal pigment epithelial depigmentation, vitreal cells, macular and disc edema, and venous sheathing. This review provides an updated summary of the current state of knowledge about birdshot retinochoroidopathy. It includes results of visual field and electrophysiological tests.

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