Among the various types of aortic arch syndromes or "pulseless disease," there is a rare primary aortitis with segmental stenosis of the aorta and great vessels, occurring predominantly in women from 20 to 35 years of age.1 These patients develop progressive narrowing of the aorta and its major branches which results in weakened pulses and low blood pressure in the distribution of the affected vessels. Pathologic examination reveals segmental thickening of the wall of the aorta and its major branches which may be associated with mural thrombosis. Histologically these vessels show a wide variety of inflammatory reactions resembling syphilis, arteriosclerosis, Buerger's disease, periarteritis nodosa, or giantcell arteritis. The condition has been referred to as Takayasu's disease, "young female arteritis,"1 and primary arteritis of the aorta.3 The etiology is unknown. Presented here is the case of a child which seems to fit this category of disease, clinically and