Introduction: Apical Hypertrophic Cardiomyopathy (AHCM) is a unique variant with distinct clinical presentation, genetics, treatment, complications and outcome. Case: A 52 year non-hypertensive Asian male presented with exertional shortness of breath for two years without chest pain, palpitation or syncope. The apex beat was heaving. Electrocardiogram revealed non q wave deep symmetrical T wave inversion in anterolateral leads and echocardiography demonstrated hypertrophied apical septum. Coronary angiogram showed normal coronaries with typical “Ace of Spade” configuration during ventriculography. Conclusion: Characterization of various forms of hypertrophic cardiomyopathy is essential for management purpose as apical hypertrophic cardiomyopathy usually have benign course.

Keywords: Apical Hypertrophic Cardiomyopathy, Hypertrophic Cardiomyopathy