Thirteen patients had a new syndrome and entity in intraocular inflammation called birdshot retinochoroidopathy, which was characterized by a white, painless eye with minimal anterior segment inflammation but particulate debris in the anterior and posterior vitreous. There was profuse retinal vascular leakage with resultant retinal, macular, and disk edema, which are the primary causes of the decreased vision in this syndrome. Long-term follow-up (mean of ten years) revealed that only two patients seemed to respond to anti-inflammatory therapy (systemic corticosteroids) and five patients progressed to 6/60 (20/200) or less visual acuity in at least one eye.