Aristolochic acid nephropathy (AAN), a progressive renal interstitial fibrosis frequently resulting in end stage renal disease, still remains a common chronic interstitial nephropathy in China. Therefore, great attention should be paid to AAN. This review summarized recent research progress of AAN in terms of in vivo aristolochic acid metabolism, epithelial mesenchymal transition, proteomics, immunity-inflammation, and autophagy, which will help to further understand the pathogenesis of AAN, and to search effective intervention targets.