Patients with left ventricular apical aneurysms represent a unique patient subgroup in the broad clinical spectrum of hypertrophic cardiomyopathy (HC) associated with the risk for sudden death, heart failure, and peripheral thromboembolism. Routine chronic anticoagulation has not been a standard recommendation for these patients. We present a 36-year-old patient with HC with features of acute coronary syndrome, likely secondary to coronary thromboembolism as a complication of left ventricular apical aneurysm. Our experience with this patient raises consideration for prophylactic anticoagulant therapy for this unusual subset of patients with HC.