Takayasu arteritis (TA), a granulomatous large vessel vasculitis involves mostly the aorta and its proximal branches and occurs most commonly in young females. The data on the epidemiology of TA is limited, probably due to the rarity of the disease. Although the disease has a worldwide distribution, it is generally thought to be much more common among Asian populations. The incidences of TA were estimated to be 1–2 per million in Japan and 2.2 per million in Kuwait. Recent epidemiologic studies suggest that TA is being increasingly recognized in Europe with reported incidence estimates varying from 0.4 to 1.5 per million. The highest ever prevalence of TA at 40 per million was estimated in Japan and the lowest ever one at 0.9 per million in US. The reported prevalences in the European populations vary between the ranges of 4.7 per million and 33 per million. These variations between the studies may be derived from geographical and genetic differences between the populations, but also may be due to the methodological differences.