Cardiac valve replacement is a rare but not exceptional eventuality in patients with relapsing polychondritis. One case requiring aortic and mitral valve replacement and its follow up is described. From the review of the literature an additional twenty patients who required cardiac valve replacement are analyzed. The mean delay between the first onset of relapsing polychondritis and operation was 6.51 years and the mean age at operation was 38.8 years. There was a preponderance of male patients (73.7%). Aortic and mitral valves were replaced in 100% and 28.5% of patients, respectively. During the four first postoperative years 23.8% of them were reoperated for periprosthetic leak or aortic aneurysm, and during the same period 52.6% died of a cardiovascular cause. Immunosuppressive agents should be employed in patients with relapsing polychondritis and cardiovascular involvement because they seem to be more effective than steroids in severe forms of the disease. Therefore, we recommend close and prolonged follow up: firstly because there can be early paravalvular prosthetic leakage due to the friability of the tissue to which it has been anchored; secondly because aortic aneurysms occur frequently in relapsing polychondritis, may be multiple, may involve all parts of the aorta and result in fatal rupture even in asymptomatic patients; and thirdly because there can be a fatal outcome due to other organ involvement, like airway obstruction, acute glomerulonephritis, or systemic vasculitis. Prophylactic composite graft replacement of the ascending aorta associated with replacement of the aortic valve and re-implantation of the coronary arteries could avoid the need for reoperation in these high risk patients.