ABSTRACT Cystathionine β‐synthase (CBS) is a pivotal catalytic enzyme in the transsulfuration pathway, widely expressed in various organs of the female reproductive system. CBS expression affects the contents of sulfur‐containing metabolites including cysteine (Cys), glutathione (GSH), and taurine. GSH and taurine act as antioxidants, participating in maintaining the cellular antioxidant defense system. In addition, several reactions catalyzed by CBS are accompanied by the production of hydrogen sulfide (H 2 S). H 2 S, as a gaseous transmitter, participates in various biological processes, including angiogenesis, inflammation, and oxidative stress. This review summarizes the structure, the distribution of CBS, factors affecting CBS expression, and CBS‐related sulfur‐containing metabolites. It also discusses the functions of CBS under normal physiological conditions and pathological circumstances in the female reproductive system and explores the possibility of the CBS/H 2 S axis as a direction in the treatment of female reproductive system diseases.