AbstractA case with relapsing polychondritis is described where primary involvement of the aortic valve cusps produced severe aortic regurgitation requiring valve replacement. An aneurysmal dilatation of the ascending aorta developing later led to disruption of the prosthesis requiring re‐operation. Superior vena caval obstruction, an abdominal aortic aneurysm which ruptured and required resection, and obstructive lesions in common iliac arteries, presumably the result of the same process that involved the aorta and the cartilaginous structures, were also see