Objective To investigate the value of electrocardiogram (ECG), MRI and cardiac ultrasound in the diagnosis of apical hypertrophic cardiomyopathy (AHCM). Methods The clinical data of 52 patients with AHCM who were admitted to the hospital from February 2015 to October 2017 were analyzed retrospectively. All patients underwent ECG, MRI and cardiac ultrasound examination. The imaging findings of AHCM were analyzed, and the effectiveness of three imaging methods in diagnosing AHCM was evaluated. Results There were 52 cases with abnormal ECG, 35 cases (67.31%) with increased left precordial RV4-6 voltage, 40 cases (76.92%) with giant negative T wave in ECG of left chest, 32 cases (61.54%) with low ST segment, no abnormal Q wave and 6 cases (11.54%) with different degrees of arrhythmia. MRI findings included simple P-apical hypertrophy, simple T-apical hypertrophy, mixed P-apical hypertrophy and mixed T- apical hypertrophy in 23 cases, 12 cases, 12 cases and 5 cases, respectively. There was apical wall diffuse hypertrophy in patients with T-apical hypertrophy, with typical black heart signs as main manifestations. There was apical wall mild hypertrophy in patients with P-apical hypertrophy, multi-segmental hypertrophy, without black heart signs. Ultrasound imaging findings mainly showed apical symmetric or asymmetric hypertrophy, uneven echo of myocardial hypertrophy, stiff contraction and narrow heart cavity or occlusion of the apex. Ultrasound examination showed that there were 22 cases without obvious abnormalities, 18 cases with apical hypertrophy, 8 cases with decreased left ventricular diastolic function and 10 cases with left atrial enlargement. Conclusion ECG, MRI and cardiac ultrasound are of certain value in the diagnosis of AHCM. MRI can accurately display the degree of myocardial wall hypertrophy, which is conductive to typing of cardiomyopathy and analyzing cardiac function. Therefore, MRI has more advantages over ECG and cardiac ultrasound in the diagnosis of AHCM.