Recent reviews have given a first overview concerning imaging techniques and clinical applications in patients with inherited, metabolic and inflammatory muscle diseases [1–4]. Given the dynamic development in this field of research, the aim of this review is to give a comprehensive overview of the available imaging techniques and present an update of clinical neuromuscular imaging applications in patients with inherited muscle diseases. Furthermore, we would like to try to give some guidelines about how and to what extent neuromuscular magnetic resonance imaging (MRI) can lead us in the right direction in terms of narrowing the differential diagnosis and making the right diagnosis prior to interventional diagnostic procedures such as muscle biopsy.