The study concerns a 44 year-old male patient with a history of intermittent episodes of rheumatic activity; the physical examination showed mitral valve stenosis and metacarpophalangeal joint deformities. The joint deformities were bilateral and symmetrical, similar to that of rheumatoid arthritis. Nevertheless, the ulnar deviation of the hands was reversible and there were no typical signs of rheumatoid arthritis in the X-ray. In view of the facts, the diagnosis of Jaccoud's Artrhopathy (JA) was made. The pathogenesis, the clinical features and the association between mitral valve stenosis and joints deformities of JA are discussed. This disease has a good prognosis and it is sparsely mentioned in the cardiologic literature. Many patients with JA are diagnosed as having rheumatoid arthritis, which is a chronic and disabling disease.