Glycogen storage disease type 1b (GSD 1b) is an ultra-rare disease that is the result of a mutation in the SLC37A4 gene and reduced function of the enzyme glucose 6 phosphate translocase (G6PT). In addition to the classic symptoms of glycogenosis type 1a (hepatomegaly, nephromegaly, hyperlipidemia, hyperuricemia, metabolic acidosis, and hyperlactatemia) patients with glycogenosis 1b also have neutropenia and impaired neutrophil function, which makes them prone to infections. Infections of the skin and mucous membranes are the most, and it is estimated that most of them develop IBD-like disease. In addition to diet and cornstarch therapy, the application of the SGLT2 inhibitor, empagliflozin, showed very good results in reduced infection and inflammation in these patients and significantly contributed to the improvement of their clinical condition and quality of life, which has already been published several times. The incidence of GSD 1b in Serbia is about 1:60,000, probably more. I will  share our experiences with diagnosis of GSD 1b, genotypic-phenotypic correlation, and results during SGLT2 inhibitor therapy, including the assessment of the patient’s quality of life.