Apical hypertrophic cardiomyopathy (AHC) is a less common type of hypertrophic cardiomyopathy (HCM). It is characterized by typical changes in ECG (deeply inverted T waves that can mimic acute coronary syndrome), echocardiography (hypertrophy of apical segments) and ventriculography (spade-like shaped left ventricular cavity). Magnetic resonance is considered as a reference method for AHC diag nosis. We present a long follow-up period of a patient with this form of HCM. The management of patients with HCM is directed at de creasing left ventricle outflow tract narrowing (if present), decreasing of intraventricular gradient (if present), heart failure treatment and prevention of sudden cardiac death. In general treatment adequate hydration is essential and diuretics should be used with caution to prevent the increase of intraventricular gradient.