Although apical hypertrophic cardiomyopathy is generally accepted as a form of hypertrophic cardiomyopathy, its underlying genetic factors, clinical course and complications may be different. The characteristics of 14 Brazilian patients with a diagnosis of apical hypertrophic cardiomyopathy are described. Symptoms were frequent and abnormal filling of the left ventricle by Doppler criteria was recorded in all patients. Rest electrocardiograms showed giant negative T waves in 5 patients, all had low exercise capacity on an exercise stress test while significant arrhythmias were detected by 24-h ambulatory monitoring in just one patient. Forty-eight first degree relatives were studied and 3 had some form of hypertrophy of the left ventricle as seen by echocardiography. Apical hypertrophic cardiomyopathy in Brazilians seems to be expressed somewhat differently from that reported in Japanese patients, since "giant' T waves are less frequent and women more involved.