We report the case of a 63-year-old female patient with apical hypertrophic cardiomyopathy, diagnosed by the presence of localized apical hypertrophy in the echocardiogram and a typical "spade like" left ventricular angiographic image, but with unique electrocardiographic features, characterized by chronic ST segment elevation, and T wave inversion, in the anterolateral leads. These changes were initially interpreted as a manifestation of acute ischemic heart disease. Chronic ST segment elevation has been occasionally described in patients with hypertrophic cardiomyopathy complicated with apical necrosis and aneurysm formation, but not in uncomplicated cases of apical hypertrophic cardiomyopathy. Its knowledge by the physician could allow avoidance of problems of differential diagnosis with more frequent heart diseases, especially acute atherosclerotic ischaemic heart disease.