The importance of clinical muscular imaging is becoming increasingly obvious in patients with suspected or proven inherited muscle diseases. Musculoskeletal US is a well-established, easily applicable and cost-effective screening technique for the detection of dystrophic changes in the striated muscle, particularly in young children. However, MRI is becoming the technique of choice in the diagnostic workup of patients with suspected hereditary muscle diseases. The role of MRI goes far beyond the pure detection of inflammatory and/or dystrophic changes. Using whole-body MRI protocols, we are able to identify muscle involvement per muscle and quantify the degree of dystrophic changes according to established rating scales leading to a description of involvement patterns. These sometimes distinct patterns of involvement can further narrow the differential diagnosis and might lead to the correct diagnosis even before invasive diagnostic procedures such as muscle biopsy.