Purpose: Apical hypertrophic cardiomyopathy (HCM) is a variant type of HCM which is known to have different characteristic compared to other types of HCM and is thought to have a good prognosis. We evaluated the characteristics of cardiac magnetic resonance imaging (CMR) and other clinical findings in apical HCM. Methods: A total of 78 consecutive subjects with apical HCM diagnosed by echocardiography who received CMR in our cardiovascular imaging center were evaluated for clinical history, delayed enhancement (DE), left ventricular (LV) mass, diastolic volume and ejection fraction (LVEF), 24-hour EKG monitoring findings, and treadmill exercise (TMT) results. Results: The mean age of the subjects was 57.26짹9.0 and gender ratio was M:F=66:12. Hypertension was present in 41 and diabetes in 13 subjects. Nine subjects presented with New York Heart Association Class II dyspnea and 11 with atypical chest pain, and the remaining 54 subjects were asymptomatic. History of syncope was present in 2 subjects. Mean LV mass index on CMR was 125.5짹34.7 g/m2, diastolic volume 136.8짹26.2 ml and LVEF 73.8짹13.2%. DE was present at the apical segments in 66 (84.6%) subjects and mean DE volume was 13.7짹14.3 ml. Apical aneurysm was present in 9 (11.5%) subjects. Among the 44 subjects who received TMT, 7 showed inadequate blood pressure response to exercise. None of the 47 subjects who received 24-hour EKG monitoring showed any evidence of ventricular tachycardia, but 9 showed evidence of atrial tachycardia and 4 showed paroxysmal atrial fibrillation. Conclusions: In apical HCM, DE on CMR is a relatively frequent finding at the apex and approximately 11% of the subjects present with apical aneurysm and 9% with inadequate hemodynamic response to exercise. According to our results, a proportion of apical HCM may need closer attention as opposed to common belief.