To compare cardiovascular magnetic resonance (CMR) characteristics between arrhythmogenic right ventricular cardiomyopathy (ARVC) patients with syncope and without syncope and explore CMR parameters related with syncope. A consecutive series of 80 patients with ARVC were divided in two groups according to history of syncope prior to CMR examinations. The biventricular function and volumes were calculated and indexed by body surface area. Fatty infiltration and late-gadolinium enhancement (LGE) were self-quantitatively analyzed according to segmental model. Patients with syncope had statistically significant greater left ventricular end-diastolic volume index (LVEDVI) (79.6 ± 23.0 vs. 69.0 ± 17.9 mL/m(2), P = 0.030), right ventricular end-diastolic volume index (RVEDVI) (122.0 ± 30.0 vs. 107.4 ± 21.8 mL/m(2), P = 0.017), and LGE incidence (52.2 vs. 21.1 %, P = 0.006) than that of patients without syncope. Patients with syncope had a trend towards greater number of segments with LGE (8.6 ± 4.2 vs. 6.6 ± 3.1, P = 0.199) than that of patients without syncope in subgroup analyses of patients with LGE, but no statistical significance was reached. Multivariate regression analysis showed the presence of LGE was independently associated with syncope in patients with ARVC (odds ratios 8.827, 95 % confidence interval 1.945-40.068, P = 0.005). CMR is helpful in detection and management of the patients with ARVC. Patients with syncope had significantly higher LVEDVI, RVEDVI and LGE incidence, and larger studies with follow-up data are needed to elucidate the relationship between LGE and syncope in patients with ARVC.