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Abstract Uveitis is a frequent (20–50%) and early feature of sarcoidosis. Typical sarcoid uveitis presents with mutton-fat keratic precipitates, iris nodules, and anterior and posterior synechiae. Posterior involvement includes vitreitis, vasculitis, and choroidal lesions. Cystoid macular edema is the most important and sight-threatening consequence. Histologic proof from a biopsy is the gold standard for the diagnosis of ocular sarcoidosis. An international workshop has recently established diagnostic criteria for sarcoidosis uveitis when biopsy is unavailable or negative: these are based on a combination of ophthalmological findings and laboratory tests. The value of recent techniques, such as PET-scan and endoscopic ultrasound-guided, fine-needle aspiration of intrathoracic nodes needs to be assessed in future studies. Corticosteroids are the mainstay treatment for sarcoidosis. Systemic corticosteroids are indicated when uveitis does not respond to topical corticosteroids or when there is bilateral posterior involvement, especially macular edema and occlusive vasculitis. In up to 15% of cases, additional immunosuppression is used, including methotrexate, azathioprine, and mycophenolate mofetil. Infliximab and adalimumab have been recently proposed for the treatment of refractory or sight-threatening systemic sarcoidosis.