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Myocardial fibrosis detected by cardiac magnetic resonance (CMR) has been reported in patients with desmin‐related myopathy, although its characteristics remain unclear. Here, we describe a case of desmin‐related restrictive cardiomyopathy wherein CMR imaging revealed myocardial oedema, ischaemia, and fibrosis in the left ventricle; the different types and processes of myocardial injury were detected by CMR. Middle wall left ventricular enhancement may be a feature of late gadolinium enhancement, and the lateral wall is often involved in cases of myocardial injury. CMR is useful for the early detection of cardiac involvement and the prediction of prognosis in patients diagnosed with desmin‐related myopathy. [ABSTRACT FROM AUTHOR]