Ocular disease occurs in approximately a third of sarcoidosis patients. The rate of disease varies around the world, with Japanese sarcoidosis patients having ocular disease in more than 70% of cases. If untreated, ocular disease can lead to permanent visual impairment, including blindness. The most common manifestation is uveitis, with anterior involvement often being self-limiting, whereas posterior involvement can be chronic. The diagnosis of ocular sarcoidosis in patients with known sarcoidosis usually requires a specific examination by an ophthalmologist. For patients presenting with uveitis of unknown etiology, criteria have been proposed for diagnosing ocular sarcoidosis. The treatment of ocular disease ranges from topical therapy to systemic treatments such as methotrexate. Recent reports have demonstrated that monoclonal antibodies blocking tumor necrosis factor can be quite effective for chronic refractory ocular sarcoidosis.