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Diagnosis and management of ocular tuberculosis (OTB) poses a significant challenge. Mixed ocular tissue involvement and lack of agreement on best practice diagnostic tests together with the global variations in therapeutic management contributed to the existing uncertainties regarding the outcome of the disease. The current review aims to update recent progress on OTB. In particular, the Collaborative Ocular Tuberculosis Study (COTS) group recently standardized a nomenclature system for defining clinical phenotypes, and also proposed consensus guidelines and an algorithmic approach for management of different clinical phenotypes of OTB. Recent developments in experimental research and innovations in molecular diagnostics and imaging technology have provided a new understanding in the pathogenesis and natural history of the disease.