During the past few years, substantial progress in the genetic diagnosis of inherited neuromuscular diseases has led to new insights into the pathological understanding of the disease. This in turn has led to the identification of numerous new genetic abnormalities associated with dystrophic or non-dystrophic disease of the striated muscles. In addition to the neurological examination and neurophysiological assessment, neuromuscular imaging is increasingly becoming an important diagnostic tool in detecting muscular involvement as well as describing the degree and pattern of involvement. Neuromuscular imaging can therefore be helpful in supporting the clinical diagnosis, limiting the complex range of differential diagnoses and guiding interventional diagnostic procedures such as muscle biopsy. Since the introduction of ultrasound in the early 1980s as a diagnostic tool in neuromuscular diseases, the dynamic field of neuromuscular imaging techniques has become more fascinating but also complex.