Retinoic acid (RA) receptor, is a group of ligand-regulated nuclear transcription factors and widely distributed in various tissue cells. The different subtypes of RA receptor can polymerize into homologous or heterogenous dimmers, and then participate in the physiological and pathological processes of embryonic development, cell proliferation and differentiation, and apoptosis. However, the relationship between the expression of RA receptor and the diseases is different in diverse cells or tissues. Recent researches demonstrated that RA receptor is closely related to the pathogenesis and development of renal diseases. This article aims to review the role of the RA receptor and its signal pathway in the development of renal diseases.