The review provides information about apical hypertrophic cardiomyopathy (HCM), which is a relatively rare phonotype of HCM. Previously, it was believed that apical HCMP has a favorable prognosis, but recent studies have demonstrated an increased risk of fatal arrhythmias and sudden cardiac death, especially among elderly patients, women and those with comorbidities such as arterial hypertension and diabetes mellitus. Patients with apical HCMP complain of chest pain, shortness of breath and palpitations. The diagnosis is based on data from various investigation methods, among which imaging techniques such as echocardiography and cardiac magnetic resonance imaging are crucial. For apical HCMP, a characteristic sign is the identification of the configuration of the left ventricular cavity at the end of the systole resembling to the type "Ace-of-spades". Treatment includes pharmacotherapy using beta-blockers, calcium channel blockers, cardiac myosin inhibitors and surgical interventions. The lack of information on unfavorable prognostic factors, the lack of specific clinical data and the complexity of instrumental diagnosis emphasize the relevance of studying the problem of apical HCMP.