PURPOSE: Birdshot retinochoroidopathy (BRC) is a rare uveitis syndrome of presumed autoimmune etiology. Therapy with systemic and periocular corticosteroids is of inconsistent efficacy, attendant with numerous potential long-term side effects. Corticosteroid-sparing strategies with agents such as cyclosporine A or azathioprine have been suggested for this disease. METHODS: We retrospectively reviewed the medical charts of patients with BRC who were evaluated consecutively at a tertiary-care, referral-based North American uveitis clinic over a 15-year period. RESULTS: Eleven Caucasian patients (22 eyes) were diagnosed with BRC, representing approximately 1% of all cases seen at the uveitis clinic. HLA-A29 was positive in all 11 patients. We elected to treat five patients with azathioprine, methotrexate, cyclosporine A, mycophenolate mofetil, and/or IvIg, as well as systemic or periocular corticosteroid injections. The median period of follow-up for the five treated patients was six years (range: 8 months-13 years). Inflammation was reduced or stabilized in five of five patients. CONCLUSION: Although the definitive strategy for the management of BRC is unknown, control of intraocular inflammation and preservation of vision is possible with corticosteroid-sparing immunosuppressive agents.