Siraitia grosvenorii (Swingle) C. Jeffrey ex Lu et Z. Y. Zhang is a unique economic and medicinal plant of Cucurbitaceae in Southern China. For hundreds of years, Chinese people have used the fruit of S. grosvenorii as an excellent natural sweetener and traditional medicine for lung congestion, sore throat, and constipation. It is one of the first species in China to be classified as a medicinal food homology, which has received considerable attention as a natural product with high development potential. Various natural products, such as triterpenoids, flavonoids, amino acids, and lignans, have been released from this plant by previous phytochemical studies. Phar- macological research of the fruits of S. grosvenorii has attracted extensive attention, and an increasing number of extracts and compounds have been demonstrated to have antitussive, expectorant, antiasthmatic, antioxidant, hypoglycemic, immunologic, hepatoprotective, antibacte- rial, and other activities. In this review, based on a large number of previous studies, we summarized the related research progress of the chemical components and pharmacological effects of S. grosvenorii, which provides theoretical support for further investigation of its biological functions and potential clinical applications.