Abstract: Prader-Willi syndrome (PWS) is a genetically imprinted disorder characterized by intellectual impairment, obesity, metabolic disorders, and behavioral abnormalities. The pathological mechanisms of metabolic disorders and behavioral abnormalities are not yet clear, and the treatment effect up to now is not satisfactory. In recent years, the role of gut microbiota in metabolic regulation has gradually been recognized. This article will systematically review the research progress of gut microbiota in PWS. Compared to healthy individuals or other simply obese individuals.PWS patients show changes in the gut microbiota. The changes are related to metabolic disorders and inflammatory status in PWS patients. Intervention of gut microbiota through diet or probiotics has played a certain role in controlling weight and improving behavioral abnormalities in PWS patients.