Purpose: To identify MRI biomarkers that could be used to follow disease progression and therapeutic efficacy in one individual muscle in patients with myotonic dystrophy type 1 (DM1). Materials and Methods: Lower limb MRI and maximal ankle dorsiflexor strength assessment, using a hand‐held dynamometer, were performed in 19 DM1 patients and 6 control subjects. The volume of residual muscle tissue of Tibialis Anterior (TA) muscle was chosen as an index for muscle atrophy, and the T2‐relaxation‐time of the residual muscle tissue was measured to evaluate edema‐like lesions. The fat‐to‐water ratio was assessed using three‐point Dixon images to quantify fat infiltration in the entire muscle. Results: The intra‐observer variability of MRI indices (∼5.2% for the residual muscle tissue volume and 2.5% for the fat‐to‐water ratio) was lower than that of the dorsiflexor torque measurement (∼11.5%). A high correlation (r = 0.91) was found between maximal ankle dorsiflexor strength and residual TA muscle tissue volume in DM1 patients. Increases in the fat‐to‐water ratio and T2‐relaxation‐time were associated with a decrease in maximal ankle dorsiflexor strength. Conclusion: MRI appears as a noninvasive method which can be used to follow disease progression and therapeutic efficacy. J. Magn. Reson. Imaging 2012;35:678‐685. © 2011 Wiley Periodicals, Inc.