Arrhythmogenic right ventricular dysplasia is a type of cardiomyopathy characterized by fibrofatty replacement of the right ventricular myocardium and associated with life-threatening arrhythmias. According to different data, arrhythmogenic right ventricular dysplasia occurs in 1 : 1000 to 1 : 5000 people. The incidence of the disease in males and females is 3:1. People over 40 are more likely to develop right ventricular heart failure as a type of arrhythmogenic right ventricular dysplasia. The criteria of the diagnosis of arrhythmogenic right ventricular dysplasia are based on the estimates of structural changes in the right ventricular myocardium, electrocardiographic changes, fibrofatty dystrophy in the course of a histological investigation, and a family history of the disease. The diagnosis can be a challenging task as there are no pathognomonic clinical or morphological markers, which makes differential diagnosis of arrhythmogenic right ventricular dysplasia with secondary myocardial pathology more difficult to perform. Objective: The diagnosis and choice of the viable surgical treatment of arrhythmogenic right ventricular dysplasia. Material and methods. After a detailed examination of a 64-year-old patient A that entailed the Holter monitoring of ECG, echocardiographic study, contrast-enhanced multispiral computed tomography and electrophysiological study resulted in the diagnosis of arrhythmogenic right ventricular dysplasia based on two major (duration of QRS complex 120 ms, reduced right ventricular myocardial contractility) and two minor criteria (inverted T-waves in V1 – V3 chest leads, a non-sustained ventricular tachycardia based on the Holter monitoring of ECG). During the programmed pacing of the right ventricle outflow tract by three extrastimuli (S1=400, S2=270, S3 = 250, and S4=200 ms) a stable paroxysm of a monomorphic right ventricle tachycardia with the cycle length 420 ms was induced which terminated spontaneously in 30 seconds. Results. Considering the obtained data on paroxymal atrial flutter, radiofrequency cardiac isthmus ablation took place as the initial treatment. As part of the follow-up treatment due to ventricular tachycardia induced during the electrophysiological study, a two-chamber cardioverter-defibrillator was implanted, a therapy was prescribed for chronic heart failure. A positive dynamics was observed through the course of the treatment. Conclusion. Predictors of negative outcome of the disease are dilatation and decreased right ventricular myocardial contractility, the left ventricle involved in the process as well as a family history of ventricular tachycardia. The effectiveness of interventive treatment of ectopic atrial and ventricular tachycardia in arrhythmogenic right ventricular dysplasia patients is low. Therefore there has been a growing tendency over the last decade to make more use of implanted cardioverter-defibrillator and less use of isolated interventive and antiarrhythmic therapy in the treatment of arrhythmogenic right ventricular dy ht ventricular dysplasia.