Apical hypertrophic cardiomyopathy is a rare form of hypertrophic cardiomyopathy (HCM) recognized by a unique spadelike configuration on the left ventriculogram. Two-dimensional echocardiography is another useful tool in the diagnosis of this condition. The diagnosis may be difficult and may mimic akinesia or apical thrombus in some patients with poor acoustic windows. A 50-year-old woman with typical angina and left ventricular hypertrophy with T wave inversion in leads V3-V6, 11, 111 and aVF is presented. Apical HCM was diagnosed with contrast-enhanced echocardiography and confirmed by finding a spadelike configuration on the left ventriculogram. Apical HCM should be considered in patients in whom symptoms and ECG findings mimic ischemic heart disease. Contrast-enhanced echocardiography is a reliable and simple method in the diagnosis of apical HCM.