AbstractA prospective study was done on 222 consectutive new patients referred to our pediatric muscle clinic to assess the diagnostic value of ultrasound imaging. Ultrasound scans were interpreted without knowledge of clinical presentation or results of other tests. Muscular dystrophy produced a brightly speckled pattern of increased echo from the muscle, whereas spinal muscular atrophy showed a moderate increase in muscle echo and associated muscle atrophy. Acute dermatomyositis produced a moderate increase in echo that varied markedly with the direction of the ultrasound beam in relation to the muscle fibres. The ultrasound scan was normal in children with hypotonia of cerebral origin, Prader Willi syndrome, ligamentous laxity, and other “nonneuromuscular” causes. In eight patients ultrasound scanning showed a striking degree of selective involvement of individual components of the quadriceps muscle, which provided considerable diagnostic help for selective needle biopsy. Ultrasound scanning in children has the major advantage of being a nonivasive and pleasant out‐patient procedure, which can be readily done on multiple sites. It is a valuable screening test in the investigation of children with neuromuscular diso