We report the case of a child with an ascending aortic aneurysm associated with aortic insufficiency. Histopathological examination of the ascending aorta and aortic valve showed findings in favor of Takayasu's arteritis, and subsequent evaluation of the entire aorta demonstrated the presence of multiple steno-occlusive lesions. This unusual clinical problem in the young population is discussed with regard to other eventual pathologies that should be taken into account in the differential diagnosis.