Apical cardiomyopathy is rare In the West. The characteristic appearance on left ventriculography has been used to confirm the diagnosis of this condition; transthoracic echocardiography can also be useful in this regard. However, apical artifacts may obscure the typical appearance during echocardiography, and although the advent of tissue harmonic echocardiography has resulted in improved image quality, the technique still may be inadequate in the establishment of a diagnosis, We hypothesized that contrast echocardiography, which improves endocardial border delineation, may be the technique of choice for the diagnosis of apical hypertrophic cardiomyopathy, We report the case of a 40-year-old woman with Down syndrome who had chest pain. The electrocardiogram showed T-wave changes in the lateral precordial leads, but cardiac enzymes were normal. Tissue harmonic echocardiography showed apical akinesia. Intravenous contrast echocardiography, however, revealed typical features of hypertrophic apical. cardiomyopathy. Thus contrast echocardiography may be used to establish the diagnosis of this condition.