Five cases are described in which aortic insufficiency developed late in the course of Reiter's syndrome. The patients had recurrent or prolonged arthritis and a high incidence of sacroiliitis, iritis and mucocutaneous manifestations. The murmur of aortic insufficiency was first noted on an average fifteen years after the clinical onset of Reiter's syndrome. Prolonged atrioventricular conduction was found in three of the patients. Microscopic examination of the aortic valve leaflets of three patients revealed little or no abnormality, although the valves of two of them were grossly incompetent due to marked dilatation of ihe aortic valve ring. Examination of the hemodynamically competent valve of one patient, who died from carcinoma of the esophagus only five months after an aortic diastolic murmur was first noted, demonstrated patchy elastic tissue disruption associated with an active inflammatory infiltrate and subendothelial collagen deposition. These changes were most marked in the root of the aorta, the aortic ring and the distal aspect of the ventricular outflow tract, and presumably cause increasingly severe dilatation and valvular incompetence as more and more of the elastic tissue support for the aortic ring is destroyed. However, inflammation of these areas may be episodic, and the prognosis in a patient with an asymptomatic murmur of aortic insufficiency may be quite good.