Apical hypertrophic cardiomyopathy (HCM) is a rare variant of HCM in the non-Japanese population (1% to 2%). Diagnostic dilemma occurs when this type of HCM is newly discovered in a patient previously diagnosed with left ventricular hypertrophy (LVH) secondary to hypertension. We describe an atypical presentation of an apical HCM in the setting of chronic hypertension (HTN) and review the literature of this rare variant of HCM. Our patient presented with chest pain and was found to have apical HCM with inducible apical ischemia and an abnormal blood pressure response to exercise. Multimodality imaging approach is proposed to evaluate the LVH when both apical HCM and hypertension are present given the impact of an accurate diagnosis on prognosis and management.