Relapsing polychondritis is an inflammatory disease that characteristically involves cartilagenous tissues. Cardiovascular involvement is a fairly common complication and the second most frequent cause of mortality in this disease. The case of a man with a progressive cardiac involvement, aortic incompetence, mitral regurgitation, and finally complete atrioventricular block offered the opportunity of reviewing the cardiovascular complications in relapsing polychondritis. The most frequent abnormalities are aortic regurgitation and aortic aneurysm. Furthermore, several cases of atrioventricular block, mitral regurgitation, and acute pericarditis have been reported. For early diagnosis and treatment of these severe complications, periodic cardiovascular examination is mandatory in these patients.