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Optimal management of childhood rheumatic diseases requires an appreciation of their multisystem nature. The eye represents an important site of involvement, and inflammation of the uveal tract is a particularly frequent and potentially debilitating extra-articular feature of some childhood rheumatic diseases. Anterior uveitis associated with oligoarticular juvenile idiopathic arthritis is an especially distinctive entity. Other disorders in children, however, can be associated with posterior and intermediate uveal tract inflammation. The potentially debilitating consequences of uveitis associated with childhood rheumatic diseases, the inadequacies of existing therapies, and the immunopathogenic basis for particular forms of uveitis have prompted the use of immunomodulatory therapy, including new biologic agents, to treat childhood uveitis. This review summarizes recent contributions to the literature that help to clarify the spectrum of conditions associated with uveitis in children, consider evidence for immunopathogenic processes associated with uveitis, and address new approaches to therapy.