The problem of the mitral valve (MV) dysfunction, both in isolation and combination forms with other anomalies and lesions of connective tissue structures, is very current. Mitral insufficiency (MI) in case of the connective tissue dysplasia is the most common valvular pathology, accompanied by different degrees of severity. Nowadays the progradient development of functional and clinical symptoms of this disease has been studied, the questions of epidemiology, diagnosis and prognosis have been clarified and reconsidered. There are important issues of correct diagnosis, time and method of treatment, the decision of usage a valve-repair or valve prosthesis in the certain clinical situation. All these practical algorithm steps depend on the clinical and surgical experience of the medical institutions which work with this pathology. The purpose of this article is to expand the knowledge of practical doctors in the issue of MI in case of connective tissue dysplasia, including the questions from the model of the MV structure to modern methods of treatment which are described in the contemporary literature.