Coix seeds, an excellent cereal resource rich in nutrients and pharmacologically active ingredients with balanced nutrition, have nice theoretical advantages for pharmacological applications in terms of hypoglycemic, hypolipidemic, antioxidant, anticancer, and antitumor activities. Its pharmacologically active ingredients are important resources for the food industry and the development of medical and health products. This review summarizes the pharmacologically active ingredients in coix seeds, a source of both food and medicine, as well as the current status of their application in medical clinics and health foods, with the aim of offering valuable information for the development and application of coix seeds in the medicinal and food fields.