Apical hypertrophic myocardiopathy (AHM) is an entity less frequently reported in our country than in Japan. We studied only three patients between 1980 and 1987. Their age varied from 26 to 40 years. Other studies included vectorcardiograms, Holter monitoring, exercise stress testing, phonocardiograms and cardiac catheterization; only one case had a genetic study, program atrial stimulation and technetium-99m pyrophosphate scintigraphy. All patients underwent echocardiographic study. The clinical features had great variations, from asymptomatic cases to the presence of important left ventricular failure and brain embolism. Electrocardiogram was characteristic in two patients; in one, cardiac catheterization showed an intraventricular pressure gradient of 19 mmHg explained by trapping of the catheter and in the other one, the end-diastole pressure of left ventricle was 22 mmHg; in both, the left ventriculogram showed the characteristic spade image. In all of them the echocardiogram showed segmentary left apical ventricular hypertrophy similar to the angiographic image; one patients had mitral regurgitation. This study demonstrates the usefulness of echocardiogram in this pathology.