We report a case of apical hypertrophic cardiomyopathy (HCM) presenting with non-sustained ventricular tachycardia. Whole-heart magnetic resonance imaging (MRI) showed normal coronary arteries and confirmed the diagnosis of apical HCM. First-pass gadolinium delayed-enhanced MRI showed local delayed enhancement in the endomyocardium in the hypertrophic lesion. The presence of perfusion defect and delayed enhancement may be a marker of a high-risk patient with apical HCM.