The authors report follow-up of a patient with noncompaction cardiomyopathy. For the first time, the patient appealed for medical care at the age of 38 years with symptoms of chronic heart failure. Echocardiography revealed dilatation of heart chambers. Left atrium dimension was 41 mm, right ventricle 37 mm, right ventricular ejection fraction — 15%, left ventricular end-diastolic dimension 50 mm, left ventricular end-diastolic volume 154 ml, left ventricular ejection fraction 16%. There were severe mitral and tricuspid regurgitation and signs of left ventricular dyssynchrony. Electrocardiography revealed complete left bundle branch blockade and hypertrophy of both ventricles. A 24-hour ECG monitoring revealed sinus rhythm, rare single polymorphic premature ventricular beats (n=296), single episode of repetitive ventricular contractions, rare supraventricular premature beats (n=120), intermittent couplets, bigeminy, paroxysm of atrial fibrillation. No laboratory signs of inflammation and cytolysis were found. Contrast-enhanced MRI of the heart revealed no abnormal contrast enhancement in early series and delayed excretion in delayed series typical for myocarditis. There were hypertrophied trabeculae and lacunae reaching compact layer of the left ventricle and typical for non-compact myocardium. Daily therapy included amiodarone 200 mg, bisoprolol 1.25 mg, spironolactone 100 mg, and then eplerenone 50 mg, rivaroxaban 20 mg, torasemide 10 mg. This approach was valuable to stabilize the patient’s condition and improve cardiac and hemodynamic parameters. Left ventricular end-diastolic volume decreased up to 136 ml, ejection fraction increased up to 55%. Thus, timely contrast-enhanced MRI of the heart made it possible to establish a rare form of cardiomyopathy, determine the correct management, as well as improve the quality of life and prognosis.