Background: Apical and midventricular hypertrophic cardiomyopathy (HCM) are rare variants of HCM, in which the hypertrophy is located mainly at the midventricular to apical levels. Heart transplantation was the only possible surgical solution for many of these patients; however, transapical myectomy represents another good alternative. We present our surgical technique of apical ventriculotomy for apical and midventricular myectomy. Technique: A 6-cm incision is made at the apex of the heart lateral to the left anterior descending coronary artery. The apical ventriculotomy provides excellent exposure of the midventricle for midventricular myectomy. The apical approach also allows access to the left ventricle for apical myectomy to enlarge the chamber. During apical myectomy, particular care is necessary to avoid injury to papillary muscles, which are often displaced with apical HCM. Secure closure of the ventriculotomy can be achieved with a double layer of suture reinforced with felt, and no complications of false aneurysm have been observed. Conclusions: The transapical approach provides excellent exposure of the apex and midventricle, and the technique is useful when myectomy is aimed at eliminating the ventricular obstruction and/or enlarging the left ventricular cavity size in patients with apical hypertrophy.