Illustrated by a case report, which was rather suggestive of coronary ischemic heart disease, the principle characteristics of apical hypertrophic cardiomyopathy are outlined. Outside East Asia, it remains a very uncommon variant of hypertrophic cardiomyopathy. The electrocardiogram in basal conditions showed giant negative T-waves (-14 mm) in the precordial leads, while the ventriculogram in RAO projection revealed the typical, so called "ace of spades" configuration. Based on the present literature and the follow-up of these patients a review of apical hypertrophic cardiomyopathy is presented. One of the most important consequences of the identification of this variant seems to be the rather good prognosis when compared to other forms of hypertrophic cardiomyopathy.