Claudin-10, a member of the claudin family of tight junction transmembrane proteins, is expressed in epithelial cells of multiple organs and tissues, with two isoforms, claudin-10a and claudin-10b. It plays a critical role in maintaining the selective permeability of cell membrane and regulating ion transport through the paracellular pathway. In recent years, claudin-10 has been reported to be associated with the development and progression of a variety of diseases. Diseases characterized by impaired function of multiple organs due to mutations in the claudin-10 gene are collectively referred to as HELIX syndrome, which generally results in clinical manifestations such as anhidrosis, renal disease, hypokalemia, xerostomia, and severe enamel wear. In this review, the research progress on claudin-10 will be presented, including its molecular structure, expression and distribution, as well as the relationship between claudin-10 function and diseases.