Takayasu's arteritis (TA) is a chronic, giant-cell vasculitis of unknown etiology, which primarily involves the aorta, its main branches and coronary and pulmonary arteries. This review focuses on the epidemiological, diagnostic, and clinical aspects of the disease, which have been changed since the first description made by Mikito Takayasu in 1908. The article also summarizes the data collected by the Italian Registry of TA in the period 1993-1998.