Takayasu's arteritis is a chronic vasculitic disease of the aorta, its major branches and the pulmonary arteries, resulting in stenosing, occlusive or aneurysmal lesion. While the precise etiology of Takayasu's disease is unknown, an autoimmune mechanism or active tuberculous inflammation have been suggested. It is more common in young oriental women but has a rare incidence in children. We experienced a case of Takayasu arteritis type IV in an 8years old girl. The diagnosis was made by physial examination and digital substraction aortography(DSA) which showed narrowing of left common carotid artery, right subclavin artery and left renal artery. The perfusion defect at posterior segment of right upper lobe was noted in lung perfusion scan. Medical treatment and percutaneous transluminal angioplasty(PTA) were performed. In addition to case report, a brief review of literature was added.