This article presents a case of diagnosis and treatment of a rare form of left ventricular (LV) hypertrophic cardiomyopathy (HCMP), asymmetric topical HCMP without obstruction of the LV outflow tract, and raises the issue of diagnosis and treatment of rare forms of this disease. In the Federal Center for Cardiovascular Surgery in 2019–2020, 5 cases of asymmetric hypertrophic apical cardiomyopathy were observed and documented with echocardiography (EchoCG), cardiac magnetic resonance imaging (MRI), and Holter ECG monitoring. A 60-year-old female patient underwent a comprehensive evaluation, including physical examination, EchoCG, MRI, Holter ECG monitoring, and coronary angiography. Apical HCMP was detected. The clinical picture was explainable from the perspective of functional and structural alterations in the LV myocardium, where full obstruction of the cardiac apex develops during systole. The small LV cavity is unable to provide effective hemodynamics. Changed small coronary arteries also contribute to this disorder, which, in results, affects myocardial oxygen supply. The administered therapy included antiplatelets, beta-blockers, angiotensin II receptor blockers, diuretics, and ranolazine. The effect of therapy was assessed as positive. Risk of sudden death, according to European Guidelines, 2014 was 0.86 %.