limb-girdle muscular dystrophies (LGMD) are a group of clinically and genetically heterogeneous diseases with progressive weakness and atrophy of pelvic and shoulder belt muscles as the main clinical features. Genetic studies have found that 29 disease-causing genes are related to this group of diseases, of which 24 genes are related to recessive LGMD, and 5 genes are related to dominant LGMD[1,2]。 Where LGMD D3 (previously known as LGMD 1G) has been shown to be caused by a mutation in the prion-like domain of the heterogeneous nuclear ribonucleoprotein D like (HNRNPDL) gene encoding an RNA processing protein[3]So far, only two families have reported in the world, from Brazil and Uruguay. Recently, through whole exome sequencing, we also discovered a third LGMD D3 family in China. The clinical characteristics of this family are different from those previously reported. The patient's limb is involved at both the distal and proximal ends, and the patient's fingers and toes are not found as previously reported. Limited flexion. The paper has beenJournal of NeurologyPublication[4]。