Background: IgA nephropathy (IgAN) is the most common primary glomerulonephritis in China. In about 20% of patients it will lead to renal insufficiency within 10 years, even in those with clinically early IgAN, which causes a great burden for patients and society. Methods: We reviewed basic and clinical research work in China. Comparisons between data from China and those from other countries were made. Results: The genetic variations conferring susceptibility to IgAN and disease progression as well as the pathogenic role of polymeric IgA1 were investigated in Chinese patients. Clinical features of Chinese IgAN with isolated microscopic hematuria, malignant hypertension, crescentic glomerulonephritis, intrarenal arterial and tubulointerstitial lesions have been characterized. Clinical trials showed that the combined therapy with urokinase + ACEI, or steroid + ACEI was more effective than with ACEI alone in reducing proteinuria and protecting renal function. The therapeutic role of MMF in IgAN remains debated, although two Chinese studies demonstrated its efficacy. Severe pneumonia can possibly occur in some cases after MMF administration for 3 months. Conclusion: IgAN remains a great challenge to Chinese nephrologists. In-depth basic studies and multicenter clinical trials are needed in the future.