Takayasu's arteritis is a rare disease that causes arterial inflammation with resulting stenosis or aneurysm of affected vessels. It primarily affects young females and has a predilection for the aorta, its primary branches, and the pulmonary arteries. Patients may present with symptoms of organ ischemia, such as stroke or claudication, or with signs of arterial stenosis, such as absent pulses or asymmetric blood pressures. Most patients require chronic immunosuppressive therapy to control inflammation. The foundation of therapy is glucocorticoids, which are often used in combination with other immunosuppressive agents, such as methotrexate, azathioprine, or mycophenolate mofetil. The goal of systemic therapy is to induce and maintain disease remission, often defined as the absence of new arterial lesions, lack of systemic symptoms, and ability to taper prednisone to less than 10 mg/d. Promising results have been seen with the use of agents directed against tumor necrosis factor in patients who have failed to maintain remission with standard therapies. Revascularization procedures such as percutaneous transluminal angioplasty (PTA) or bypass grafting are reserved for patients who have critical organ ischemia or severe extremity claudication. Bypass grafting has a lower restenosis rate than PTA and is the preferred method of restoring blood flow to affected organs. Frequent laboratory testing is recommended to monitor for drug toxicity and signs of disease relapse. At each clinic visit, physicians should auscultate for bruits over primary arterial branches and evaluate for asymmetry between arm and leg blood pressures. Biannual radiographic imaging with magnetic resonance or CT angiography is helpful to examine for evidence of new aortic aneurysms or arterial stenoses.