Polycystic ovary syndrome (PCOS) is a common and frequent disease and always leads to endocrine and metabolic disorders among women of reproductive age. In most PCOS patients, the serum level of anti-mullerian hormone (AMH) is significantly higher than that of normal women of childbearing age. AMH is an important auxiliary diagnostic method for PCOS. AMH may play an important role in the occurrence and development of PCOS and potentially affect the success of in vitro fertilization (IVF) treatments, although the exact mechanisms remain unclear. This review focuses on the relationships and mechanisms of dysregulated AMH and follicular development in PCOS based on recent research. It is mainly manifested in the regulation of related signal pathways, the negative feedback regulatory loop of several gonadal hormones such as follicle-stimulating hormone, luteinizing hormone, estradiol, and testosterone, as well as the influence of AMH on IVF in PCOS patients. Additionally, it puts forward some suggestions, which will provide some helpful ideas or directions for future further research on PCOS.