While sarcoglycan deficiencies (LGMD2C, D, E and F) are relatively rare, dystrophinopathies [type Duchenne (DMD) and type Becker (BMD)] are the most common muscular dystrophies worldwide. Therefore, late onset BMD patients or sporadic symptomatic female carriers of DMD often enter the differential diagnosis in sporadic LGMD patients. Furthermore, recently published studies emphasised an overlap between LGMD2I and the group of dystrophinopathies mainly as both often have calf or generalised muscular hypertrophy, and respiratory and cardiac involvement [38, 39]. However, on muscle imaging all our patients with BMD and alpha-sarcoglycanopathy showed pronounced signal changes in the anterior rather than the posterior thigh muscles (as present in LGMD2I patients). In accordance with our results, a predominant affliction of the anterior thigh compartment has been observed in dystrophinopathy and LGMD2D [47–49]. Thus, the relation between knee extensor and flexor involvement might be useful in distinguishing dystrophinopathies and sarcoglycan deficiencies from LGMD2I on muscular MRI. Finally, alpha-sarcoglycanopathy can be differentiated from BMD patients by the greater extent of upper limb involvement [47] and by the different pattern in the lower limbs on muscular MRI [50].