Purpose of reviewTakayasu arteritis has long been considered as an uncommon disease, rather specific to the Far-East; however recent surveys show that the disease can be seen in all ethnicities around the world with increasing prevalence rates. Nowadays, it would not be fair to consider Takayasu arteritis as a rare disease.Recent findingsAlthough involvement of the thoracic aorta and its branches was more common among females, males had a tendency toward limited involvement of the abdominal aorta and its branches. Pregnancy seems to cause serious risks for both maternal and fetal health. Takayasu arteritis frequently coexist with inflammatory bowel diseases and the two diseases may have common genetic background and molecular pathways. New imaging tools such as computerized tomography or magnetic resonance angiography, fludeoxyglucose positron emission tomography-computerized tomography and recently contrast-enhanced ultrasonography are frequently used in the diagnosis and to assess vascular inflammation. Accumulating evidence shows that biological agents such as anti-tumor necrosis factor agents, tocilizumab and rituximab could be used effectively in refractory cases. The restenosis or occlusion risks are still high with vascular interventions. The mortality seems to be decreasing in recent years.SummaryThe ethiopathogenesis of the disease should be clarified. Our feature goals should be to discriminate better active inflammation from quiescent disease enabling more effective treatment.