Abstract: As a member of steroid/thyroid hormone receptor superfamily, chicken ovalbumin upstream promoter transcription factor II(COUP-TFII)is an orphan receptor since its ligand has remained unknown. It has been established that COUP-TFII participates in many pathophysiological processes of organic development & differentiation, metabolic regulations, regulating angiogenesis & lymphangiogenesis, carcinogenesis or suppressing cancerous proliferation & metastasis in different tumor microenviron ments. Distributed mostly in glomerulus, distal tubule, collecting tubule and Bowman capsule in kidney, COUP-TFII modulates renal formation as well as initiation and progression of various renal diseases. This review summarized the latest advances of COUP-TFII in renal development, renal tumor, acute renal injury and renal fibrosis.