Two patients in whom ocular Lyme disease was suspected and who had antibodies to Borrelia burgdorferi developed birdshot chorioretinopathy and carried the HLA-A29 antigen. In a series of 11 patients with birdshot chorioretinopathy who carried the HLA-A29 antigen, three patients had antibodies against B. burgdorferi as determined by either immunofluorescence assay, enzyme-linked immunosorbent assay, Western blot analysis, or a combination of these tests. Further studies will be necessary to evaluate whether this is a false-positive reaction or whether B. burgdorferi has a causative role in the pathogenesis of birdshot chorioretinopathy.