Hypertrophic cardiomyopathy (CMH) is defined by the absence of left ventricular dilatation and the presence of myocardial hypertrophy that is not due to another recognised cause of hypertrophy such as systemic hypertension or aortic stenosis. The bizarre histological appearance of the myocardium is the hallmark of CMH. Myocytes and bundles of myocytes are malaligned and fibrosis may be extensive. Disarray is maximal in areas of macroscopic wall thickening. The condition is most easily recognized in a series of transverse, short axis slices across both ventricles. Histological examination of sections taken in this transverse plane at all three levels (high, mid, apical) including septum, anterior, posterior and lateral walls are needed. The authors report a case of cardiac sudden death to a 45 years old white man. The diagnosis was revealed intra vitam and it was confirmed post mortem.