The authors report the anatomo-clinical features of aortic insufficiency complicating atrophic polychondritis, a rare inflammatory disease affecting mainly cartilaginous tissues. This case illustrates the inflammatory changes of the aortic wall, particularly progressive during this disease, responsible for aortic insufficiency and aneurysmal dilatation of the ascending aorta which required aortic valve replacement and prosthetic replacement of the ascending aorta. Histological analysis showed inflammatory lesions of the aortic wall comparable to the cartilaginous lesions described in this condition and suggesting a common physiopathogenic mechanism.