MRI is increasingly being used in the evaluation of patients with suspected or proven inherited or metabolic neuromuscular disorders. MRI provides a high soft tissue contrast allowing excellent assessment of striated muscles concerning shape, volume (hypotrophy, hypertrophy) and tissue architecture [1, 2]. Because of the lack of ionising radiation, MRI has become a valuable imaging method in children, although sometimes sedation might be necessary. Basically, MRI is performed as a multi-sequence imaging protocol including T1-weighted (T1W) and T2-weighted (T2W) (turbo) spin echo as well as fat-suppressed (short tau inversion recovery or spectral fat suppression techniques) T2-weighted sequences (T2WFS). The image acquisition is performed in the axial plane with a slice thickness of 5-7 mm. If necessary, additional images in other anatomical planes (coronal, sagittal) can be easily acquired.