Takayasu's arteritis is a chronic inflammatory disease of unknown etiology involving the thoracic and abdominal aorta and its major branches. In some cases other vessel such as renal arteries, coronary arteries, and even pulmonary arteries may be involved. Total aortography is very important, because the clinical features are determined by the extent and severity of the specific artery involved in the occlusive phase of the disease. We report a case of Takayasu's arteritis type IV in a 38 year man with pulmonary arterial involvement and pulmo-nary hypertension.