Here we report a 3-year and 7-month-old boy with a muscular ventricular septal defect at the apex. At age 7 months, pulmonary artery banding was performed. Surgical treatment consisted of double patch closure via septal ventriculotomy and a main pulmonary artery plasty by an end-to-end anastomosis with cardiopulmonary bypass. There was no residual leak of the ventricular septal defect or ventricular aneurysm and the postoperative course was good. This technique could be a useful surgical option for repair of apical muscular ventricular septal defects.