A 79-year-old man was admitted for a transcatheter aortic valve replacement due to severe aortic stenosis. A preoperative chest computed tomography with angiography revealed an apical variant hypertrophic cardiomyopathy with a prominent apical pouch. In addition, there was near-complete obliteration of the left ventricle in the mid to apical aspect during systole suggesting a midventricular gradient. Postoperative transthoracic echocardiography confirmed the apical variant hypertrophic cardiomyopathy with an apical aneurysm and a gradient with a peak velocity of 2 m/s, and mid-cavitary gradient with a peak velocity of 3 m/s. It also revealed a fusiform aneurysmal dilatation of the ascending aorta.