Cardiomyopathy is considered one of the main causes of heart failure and sudden cardiac death, at least in young people. Approximately 50% of patients who die suddenly in childhood or adolescence or undergo heart transplantation suffer from this condition. The purpose of this literature review is to study and highlight the issues of etiology, pathogenesis, clinical features, diagnosis and treatment of hypertrophic cardiomyopathy from the point of view of modern ideas. The search and analysis of domestic and foreign literature materials using the PubMed and eLibrary databases was carried out. Of particular interest is the etiology of primary congenital cardiomyopathies, in respect of which research continues. As a result of the implementation of genetic factors, multiple structural and functional changes in the myocardium develop, which lead to changes in hemodynamics. Cardiomyopathy is a clinically heterogeneous disease, and one of the factors that determine the clinical phenotype is the genotype. In addition to standard laboratory testing, patients with suspected hypertrophic cardiomyopathy are advised to undergo medical genetic counseling to identify the causative mutation, and often to obtain prognostic information. The fundamental imaging method is echocardiography, but the role of magnetic resonance imaging in the diagnosis of the disease is also considered. Patients with symptomatic obstructive hypertrophic cardiomyopathy are usually recommended first-line pharmacotherapy with -blockers or non-dihydropyridine calcium channel blockers. Currently, research on new drugs for the treatment of hypertrophic cardiomyopathy inhibitors of cardiac myosin is ongoing. Surgical methods of treatment are developing progressively, however, methods of conservative treatment require further active research of drugs that have not been used before.