Takayasu arteritis (TA) is a large vessel vasculitis that involves the aorta, its major branches and pulmonary arteries. Diagnosis of TA during childhood remains challenging due to the non-specific symptoms. We report a fouryear- old girl presenting with fever, fatigue, weight loss, and elbow pain who was later diagnosed as childhood TA. On admission, she had fever, hypertension, decreased pulses, bruits, hepatosplenomegaly, and increased erythrocyte sedimentation rate and C-reactive protein level. Computed tomography angiography showed luminal narrowing and wall thickening in ascending aorta, brachiocephalic, left common carotid and left vertebral arteries and descending aorta. Oral corticosteroid (prednisone, 2 mg/kg/day) was instituted, later followed by oral methotrexate (12.5 mg/m(2)/week). TA is rare in children; however, childhood TA must be considered in children who present with non-specific systemic symptoms, hypertension and increased acute phase reactants.