Duvoglustat, an alkaloid azasugar or iminosugar, is a biologically active natural compound that exists in mulberry leaves and Commelina communis (dayflower) as well as from several bacterial strains such as Bacillus and Streptomyces species. Duvoglustat is an investigational pharmacological chaperone for the treatment of acid α-glucosidase (GAA) deficiency, which leads to the lysosomal storage disorder Pompe disease, which is characterized by progressive accumulation of lysosomal glycogen primarily in heart and skeletal muscles. Duvoglustat possesses antihyperglycemic, anti-obesity, and antiviral features. Most importantly, pre-meal intake of duvoglustat in therapeutic concentration has resulted in the inhibition of postprandial hyperglycemia and hyperinsulinemia. Thus, duvoglustat seems to be a potential treatment for checking or setting back the inception of diabetes. No duvoglustat-related adverse events or drug-related tolerability issues were identified in phase II clinical trial for the treatment of Pompe disease.