The concentric fatty infiltration pattern is reportedly rare in other neuromuscular disorders, including alpha-sarcoglycanopathy [11], dystrophinopathy with a clinical phenotype overlapped with LGMD2I [11, 27], collagen VI-related myopathy [28], laminopathies [29], RYR1-related myopathies [30], congenital muscular dystrophy with rigid spine syndrome [31, 32], Emery-Dreifuss muscular dystrophy caused by mutations in LMNA [33, 34], dysferlinopathy [35], neutral lipid storage disease with myopathy caused by mutations in PNPLA2 [36], and inflammatory myopathy with anti-SRP antibodies [37]. Here, PNPLA2 is linked to rigid spine syndrome.