We present a patient with asymptomatic apical hypertrophic cardiomyopathy (AHCM) who recently developed cardiac arrhythmias, and shortly discuss the diagnostic modalities, differential diagnosis, and treatment strategy for this condition. AHCM is a rare form of hypertrophic cardiomyopathy, which usually involves the apex of the left ventricle. AHCM can occur with varied presentations such as chest pain, palpitations, dyspnea, syncope, atrial fibrillation, myocardial infarction, embolic events, ventricular fibrillation, and congestive heart failure. The most peculiar electrocardiogram findings are giant T-waves inversion in the precordial leads with left ventricular (LV) hypertrophy. A transthoracic echocardiogram is the initial diagnostic modality in the evaluation of AHCM and shows hypertrophy of the LV apex. Other diagnostic modalities, including left ventriculography, multislice spiral computed tomography, and cardiac magnetic resonance imagings, are also valuable tools. Medications used to manage include verapamil, beta-blockers, and antiarrhythmic agents. An implantable cardioverter defibrillator (ICD) is recommended for high-risk patients.