Apical hypertrophic cardiomyopathy is a recently defined subgroup of primary hypertrophic cardiomyopathy. Ten new cases are studied. The electrical features (anomalies of repolarisation) were constant and giant negative T waves were observed in 3 out of 10 cases. Two-dimensional ultrasonography confirmed the diagnosis in every case; the optimal views consisted of 4 cavity scans obtained by the apical approach. When angiography was performed (7 cases out of 10), it confirmed the ultrasonographic findings and was able to eliminate any coronary artery lesions. The patients had a mean age of 48.3 years with a marked male predominance (sex ratio of 0.8) and all of the patients are alive with a mean follow-up of 3.4 years. The functional handicap was moderate (class III: 1 case, class II: 5 cases and class I: 4 cases) and did not progress during the period of follow-up. The family surveys performed revealed that apical hypertrophy can be integrated into the spectrum of segmental hypertrophic cardiomyopathy. In two cases, this survey revealed the coexistence in the same family of segmental hypertrophy with different topography. The long term prognosis of this disease is unknown and the authors consider that the risk of ventricular arrhythmia justifies routine rhythmological evaluation in order to guide subsequent treatment.