Schizoiridoid glycosides are secondary metabolites produced in plants with multiple biological activities such as liver protection, anti-inflammatory, hypoglycemic and blood lipids, and are widely used in clinical practice. Based on the research progress on the biosynthesis pathway of schizoiridoid glycosides and the mining and regulation mechanism of key enzyme genes at home and abroad in recent years, this paper mainly reviews the research progress of the biosynthesis pathway of schizoiridoid glycosides in medicinal plants, key enzymes (GPPS, GES, G10H, 8HGO, IS, IO, 7DLGT, DL7H, LAMT, SLS) and coding genes. It provides a basis for further elucidating the mechanism of its biosynthesis pathway and the regulation of key enzymes, improving the accumulation of effective active ingredients, and easing the tension of wild resources of medicinal plants.