Takayasu arteritis, a rare granulomatous vasculitis affecting young people, is associated with considerable morbidity and premature mortality. In most patients the diagnosis is delayed until after the development of substantial arterial injury. Studies of noninvasive imaging techniques suggest that these approaches might facilitate earlier diagnosis and have a role in monitoring disease progress; however, they remain limited in their ability to accurately quantify inflammatory disease activity in the arterial wall. A lack of controlled clinical trial data complicates the choice of therapy for Takayasu arteritis, and clinical indices for monitoring disease activity are currently suboptimal. Increasing knowledge of the pathogenesis of the large vessel vasculitides might eventually lead to novel targeted therapies. Preliminary data from open-label trials of anti-tumor necrosis factor therapy are encouraging, but there is an urgent need for controlled clinical trials to establish optimum therapeutic approaches for this disease. These trials should include a prospective assessment of the use of noninvasive imaging modalities in the diagnosis and subsequent management of Takayasu arteritis.