The clinical features, CMR characteristics and outcomes of arrhythmogenic left ventricular cardiomyopathy (ALVC), which is a very rare nonischemic cardiomyopathy, are currently not well studied. The purpose of the study is to investigate the clinical and cardiovascular magnetic resonance (CMR) imaging characteristics of arrhythmogenic left ventricular cardiomyopathy (ALVC). Fifty-three consecutive patients with ALVC were divided into two groups: ALVC patients without right ventricular (RV) involvement (n = 36, group 1) and those with RV involvement (n = 17, group 2). Clinical symptoms, cardiac electrophysiological findings, and CMR parameters (morphology, ventricular function, and myocardial fibrosis and fatty infiltration) were evaluated in both groups. The two groups showed no significant difference in age, gender, or presenting symptoms (P > 0.05). Right bundle branch block ventricular arrhythmia was less common in patients without RV involvement (50.0% vs.64.7%, P = 0.031). There were no significant differences in left ventricular function between the two groups, however right ventricular ejection fraction was significantly lower in group 2 (40.1 ± 4.0% vs. 48.7 ± 3.9%, P < 0.001). Inverse correlations of left ventricular ejection fraction with fat volume (r = -0.883, p = 0.001), late gadolinium enhancement (LGE) volume (r = -0.892, 0.013), ratio of fat/LGE (r = -0.848, p < 0.001), indexed left ventricular end diastolic volume (r = -0.877, p < 0.001) and indexed left ventricular end systolic volume (r = -0.943, p < 0.001) were all significant. ALVC is a rare disease with fibro-fatty replacement predominantly in the left ventricle, impaired left ventricular systolic function, and ventricular arrhythmias originating from the left ventricle. ALVC with right ventricular involvement may have a worse prognosis.