The aortic arch syndrome characterised by diminished or absent pulses in the arteries arising from the aortic arch has many etiologies, such as syphilitic aortitis, dissection of the aorta, atherosclerosis and Takayasu's arteritis. The diagnostic criteria of Takayasu's arteritis often do not mention histological investigations. We observed a case of an aortic arch syndrome, presenting as a classical case of Takayasu's arteritis but caused by a severe form of atherosclerosis.