Takayasu arteritis (TA) is a chronic nonspecific granulomatous vasculitis affecting aorta and its main branches, coronary and pulmonary arteries. TA often occurs in young women and has a characteristic heterogeneity depending on ethnicity and geographical location. Although the pathogenesis of TA remains unclear, the interaction of many factors, such as autoimmunity, inflammation, genetic and environmental factors and so on, is involved in the occurrence and development of TA. Angiography, which is recognized as the gold standard in evaluating vascular lesions in TA, combined with computer tomography angiography (CTA), magnetic resonance angiography (MRA), ultrasonography, (18)Fluorodeoxyglucose positron emission tomography (F-18-FDG-PET) could not only provide important information for early diagnosis but also detect disease activity, and thus further guide the treatment in TA. In addition, beside the commonly used corticosteroids, immunosuppressive agents, percutaneous transluminal angioplasty (PTA) and surgical revascularization, anti-tumor necrosis factor (TNF) agent has been more widely used in refractory cases of TA. The objective of this review is to systemically describe the pathogenesis, clinical characteristics, diagnosis, treatment and prognosis of TA.