The article presents a clinical case of a combination of mitral valve prolapse (MVP), mitral annular disjunction (MAD), and ventricular arrhythmia. The presence of MAD worsens the prognosis in MVP and predisposes to life-threatening ventricular arrhythmias. In a 42-year-old patient, MAD was detected during echocardiography to determine the indications for surgical correction of mitral insufficiency in MVP. Severe myxomatous degeneration of the mitral valve leaflets, polysegmental prolapse, and typical auscultatory pattern (systolic click followed by systolic murmur in the second half of systole) were the indications for the targeted search for MAD. Multi-day (ECG) monitoring recorded nonsustained ventricular tachycardias and premature ventricular complexes (PVCs). Cardiac magnetic resonance imaging was performed for confirmation the diagnosis and searched for left ventricular myocardial fibrosis accompanying MAD. Finally, MAD was confirmed, but myocardial fibrotic changes were not detected. Owing to the absence of myocardial fibrosis, the patient was treated conservatively with a beta-adrenoblocker (25 mg/day slow-release metoprolol succinate) in combination with 25 mg/day allaforte. Repeated 24-h ECG monitoring did not detect ventricular tachycardias and nonsustained registered a significant decrease of number of PVCs. The patient is followed up prospectively due to high risk factors for fibrosis and worsening prognosis, which may require surgical correction of the existing disturbances and/or implantation of a cardioverter-defibrillator.