Abstract: With high biological activity, sphingosine 1-phosphate (S1P) is also a potential therapeutic target for various diseases. It participates in many physiological processes such as cell proliferation, apoptosis and metabolism. Sphingosine kinase-1 (SphK1) is a key regulatory enzyme of maintaining the level of S1P. It is involved in the occurrence and development of cancer, infection and inflammation. It has been confirmed that SphK1 and S1P receptors are expressed in kidney and are associated with the occurrence and development of various kidney diseases. Therefore the roles of SphK1 and S1P in kidney diseases have attracted growing attention. This review summarized the research status of the role of SphK1/S1P signaling pathway in renal diseases, focusing upon SphK1/S1P signaling pathway itself and its causative roles in various renal diseases.