OBJECTIVES: Takayasu arteritis is a rare large vessel vasculitis of unknown etiology, in which both early diagnosis and follow-up present very significant challenges. The high incidence of disease-associated morbidity and significant risk of premature death-particularly in young adults-mandate the need to facilitate early diagnosis and aggressive treatment where appropriate. The aim of this review is to summarize the current level of knowledge regarding the usefulness of evolving imaging modalities in the diagnostic workup and management of patients suffering with Takayasu arteritis. We also propose an imaging algorithm for the evaluation of this population. METHODS: A MEDLINE search for articles published between January 1999 and December 2011 was conducted using the following keywords: Takayasu arteritis, imaging modalities, echocardiogram, cardiac magnetic resonance, positron emission tomography scan, diagnosis. RESULTS: Imaging studies-particularly cardiac magnetic resonance-can assist early diagnosis by demonstrating vascular lesions even when angiography is negative, by identifying the presence of vascular inflammation and/or wall thickening; they are also useful for monitoring purposes. However, availability, expertise, high cost, and radiation are considerable limitations. Magnetic resonance imaging, although it can detect both anatomic and pathophysiologic changes without radiation, is time-consuming, needs high expertise, and still remains an expensive tool, not widely available. CONCLUSIONS: Knowledge of the advantages and limitations of the various imaging procedures can complement the physicians' clinical assessment and, along with nonspecific serologic tests, can aid them in diagnosing active arteritis and commence relevant treatment early on, as well as monitor activity and tailor therapy subsequently.