We report a case of a 49 year-old woman suffering from severe aortic regurgitation due to Takayasu's disease involving the ascending and abdominal aorta and the brachial arteries. The patient was managed successfully with aortic valve replacement. Considering the Literature data, the pathogenesis of the aortic insufficiency and the main surgical and anaesthesiologic problems related to the Takayasu's disease are discussed.