Takayasu arteritis is a vasculitis of aorta and large vessels and it is rarely diagnosed in childhood in Turkey. An 11-year old girl was admitted for the manifestations of hypertensive cardiomyopathy and congestive heart failure. As plasma renin activity was found to be elevated digital substruction angiography was pertormed and segmentary stenosis, poststenotic dilatation and anevrysms of great arteries including aorta and renal arteries were detected leading to the diagnosis of Takayasu arteritis. Besides immunosupressive and antituberculous therapy, autotransplantation was performed to one kidney of the patient with a successfull result. We present this case in order to review the clinical and laboratory findings and the management of Takayasu arteritis.