Jade-1 is originally identified by the yeast two-hybrid system as a protein partner of von Hippel-Lindau (pVHL) tumor suppressor, a well-known renal tumor suppressor. In cellular signaling pathways, many upstream Jade-1 regulators, such as pVHL, CK1α, PC1, and NPHP4, can control its activity by stabilization, phosphorylation, and nuclear translocation. Numerous downstream effectors, including β-catenin, AKT, p21, and Bcl-2, are well modulated by Jade-1, which mainly regulates cell proliferation and apoptosis. Jade-1 is also deemed to be a candidate of transcriptional co-activator associated with histone acetyltransferase (HAT) activity. This review focuses on the anticancer role of Jade-1 in clear cell renal carcinoma and the inhibitory effect of Jade-1 on cystic renal diseases. This review aims to provide a basis of disease prevention or therapy.