Right ventricular dysplasia, also named right ventricular cardiomyopathy, cardiac lipidosis and partial absence of the right ventricular myocardium, is an uncommon disease in which the myocardium is partially replaced by fat and fibrous tissue. The clinical picture is usually characterized by complex arrhythmias, causing sudden death in adolescents and young adults, and cardiac failure. In this report, an association between this disease and "Ebstein-type" tricuspid valve malformation has been diagnosed in a 22 year old female patient with cianosis and dyspnea on exertion.