Dermatomyositis (DM) and muscular dystrophy are clinically difficult to differentiate.To confirm the feasibility and assess the accuracy of conventional magnetic resonance imaging (MRI), T2 map, diffusion tensor imaging (DTI), and diffusion kurtosis imaging (DKI) in the differentiation of DM from muscular dystrophy.Forty-two patients with DM proven by diagnostic criteria were enrolled in the study along with 23 patients with muscular dystrophy. Conventional MR, T2 map, DTI, and DKI images were obtained in the thigh musculature for all patients. Intramuscular T2 value, apparent diffusion coefficient (ADC), fractional anisotropy (FA), mean diffusivity (MD), and mean kurtosis (MK) values were compared between the patients with DM and muscular dystrophy. Student's t-tests and receiver operating characteristic (ROC) curve analyses were performed for all parameters. P values < 0.05 were considered statistically significant.The intramuscular T2, ADC, FA, MD, and MK values within muscles were statistically significantly different between the DM and muscular dystrophy groups (P<0.01). The MK value was statistically significantly different between the groups in comparison with T2 and FA value. As a supplement to conventional MRI, the parameters of MD and MK differentiated DM and muscular dystrophy may be valuable. The optimal cut-off value of ADC and MD values (with respective AUC, sensitivity, and specificity) between DM and muscular dystrophy were 1.698 ×10-3mm2/s (0.723, 54.1%, and 78.1%) and 1.80 ×10-3mm2/s (61.9% and 70.2%), respectively.Thigh muscle ADC and MD parameters may be useful in differentiating patients with DM from those with muscular dystrophy.