Selenium-binding protein 1 (SELENBP1) is a selenium-containing protein localized in the cytoplasm and nucleus discovered in 1989. Previous studies have shown that SELENBP1 plays an important role in protein transport in Golgi, ubiquitination/deubiquitination-mediated protein degradation, sulfur metabolism, and regulation the stability of hypoxia-inducible factor (HIF). It is also closely related to the development of diseases such as halitosis, cancer, schizophrenia and kidney damage. In this paper, the recent research progress on the biological function of SELENBP1 and its relationship with disease is reviewed and prospected.