During the past 5 years an abundant amount of data has been published concerning the degree and pattern of striated muscle involvement in inherited muscle disease. These data have led to a new era in the non-invasive diagnosis of neuromuscular diseases. The diagnostic flowcharts presented in this review are based on these data collected during the past few years. However, this is just a beginning, and we have still a long way to go. Continuously new genetic disease entities leading to muscle dystrophy can be identified and described by using new diagnostic tools. Therefore, the role of neuromuscular imaging has to be considered as a dynamic field of research that will gain even more importance in the near future. While the first imaging approaches focussed mainly on certain muscle groups primarily of the lower limbs, current and future imaging protocols should consider a “whole-body approach” such as whole-body muscle MRI including the muscle of the trunk, shoulder girdles and upper extremities. This can extend the possibilities for pattern description and recognition.