Current evidence suggests cardiac involvement and electrocardiographic changes of increasing frequency with age in patients with myotonic dystrophy type 2 (DM2). Myocyte hypertrophy with concurrent fibrosis seems to be the anatomical correlate. Moreover, morphological and functional changes indicative of subclinical cardiomyopathy have been demonstrated by means of cardiac magnetic resonance imaging (CMRI) and spectroscopy in patients with no overt cardiac disease. We present a 68-year-old woman with genetically established DM2 and no clinical, electrocardiographic or echocardiographic signs indicative of cardiac involvement. CMRI revealed delayed contrast enhancement of the anterior portion of the interventricular septum, indicating myocardial involvement. Contrast-enhanced CMRI might be a useful diagnostic tool in assessing cardiac involvement in cases of DM2. The role of delayed contrast enhancement should be further investigated in order to elucidate the cardiac features of this fascinating multisystem disease.