Apical hypertrophic cardiomyopathy (HCM) is a rare morphological variant (1—10%), in which hypertrophy is localized primarily in the apex of the left ventricle (LV). Patients with apical HCM have progressive intractable diastolic heart failure with severe symptoms that result from reduced LV volume and cardiac output. For a long time, the only treatment option for this form of hypertrophy in adults was heart transplantation. Currently, transapical myectomy in adults has proven itself to be an effective method adequately increasing LV cavity and cardiac output in early and late postoperative period. For pediatric population, there is a little information on the treatment of apical HCM, and heart transplantation is preferred in most cases. This clinical case describes the first successful experience of transapical myectomy in a child with apical type of HCM in the Russian Federation.