Apical hypertrophic cardiomyopathy was defined initially by three electrocardiographic and angiographic criteria: the presence of giant (over 10 mm) inverted T waves in leads V4 and V5 of the resting ECG; an "ace of spades" appearance of left ventricular angiography in end diastole in the right anterior oblique projection; the electrocardiographic sum RV5 + SV1 greater than 35 mm. There after, authentic cases of apical hypertrophy have been demonstrated by imaging techniques or observed anatomically without the presence of these three criteria. The authors review the epidemiological, clinical and paraclinical features of this particular form of hypertrophic cardiomyopathy.