Abstract: As an evolutionarily highly conserved signaling transduction pathway, Hippo signaling controls cell proliferation, apoptosis and migration, regulates organ growth and tissue repair and induces carcinogenesis. Under normal circumstances, Hippo pathway maintains normal development and homeostasis of renal tissue. During continuous stimulations of external factors, the pathway mediates the development of renal disease through mediating renal tissue injury and repair, renal cell proliferation and apoptosis. This review summarized the roles of this pathway in acute renal injury, chronic kidney disease, polycystic kidney disease, diabetic nephropathy, renal cell carcinoma and other diseases. Also the related pathway inhibitors were also discussed for identifying new therapeutic targets and strategies.