Hypertrophic cardiomyopathy (HCM) is characterized by left ventricular hypertrophy in the absence of abnormal cardiac loading conditions. Apical hypertrophic cardiomyopathy (Yamaguchi syndrome) is a relatively rare HCM phenotype. It is characterized by apical left ventricular hypertrophy, often with the absence of left ventricular outflow tract obstruction. In patients with HCM symptoms of angina are quite common, mostly due to microvascular dysfunction or oxygen supply demand mismatch. Here we present a case of apical hypertrophic cardiomyopathy with concomitant severe coronary artery disease.