Midventricular hypertrophic cardiomyopathy is a rare form of cardiomyopathy that may be associated with an apical aneurysm. The mechanism of aneurysm formation is uncertain, but it may be related to subendocardial ischemia. In this report, we describe a 57-year-old man with recurrent ventricular arrhythmias that were refractory to medical treatment because of midventricular hypertrophic cardiomyopathy and apical aneurysm. He was treated successfully with apical aneurysmectomy, myectomy, and subendocardial resection. Six months postoperatively, the patient was free of symptoms and was taken off all anti-arrhythmic medications with one inappropriate discharge from the implantable cardioverter-defibrillator at 4 months.