Abstract： Glycogen storage disease type Ⅰb（GSD Ⅰb）is an autosomal recessive genetic disease characterized by abnormal glycogen metabolism.The clinical manifestations include signs of metabolic abnormality of the typical GSD I，and the patients with GSD Ⅰb also have neutropenia and symptoms including recurrent infection，oral and intestinal mucosal ulcers，inflammatory bowel diseases and autoimmune thyroiditis.However，the detailed mechanism of GSD Ⅰb has not been clarified.Treatment with sodium-dependent glucose transporters 2（SGLT2）inhibitors improves both neutropenia and neutrophil function through reducing 1，5-anhydroglucitol（1，5-AG）in plasma，as well as alleviates the related clinical symptoms，so SGLT2 inhibitors are increasingly used in the treatment of GSD Ⅰb.Here it reviews the neutropenia mechanism and recent advances in animal-based research and clinical application of SGLT2 inhibitor therapy for GSD Ⅰb.