BACKGROUND The most appropriate management of Henoch-Schönlein Purpura (HSP) nephritis with nephrotic-range proteinuria remains uncertain. The aim of this study was to evaluate the clinical therapeutic effects of mycophenolate mofetil and low-dose steroid in Henoch-Schönlein purpura nephritis (HSPN) with nephrotic-range proteinuria and pathological classification less than IV in children. MATERIAL AND METHODS The clinical effects of MMF and low-dose steroid therapy were studied in children with Henoch-Schönlein purpura nephritis manifested with nephrotic-range proteinuria, normal kidney function, and <50% crescents or sclerosing lesions on renal biopsy. We enrolled 32 boys and 29 girls with nephrotic-range proteinuria, normal kidney function, and pathological classification less than IV on renal biopsy. We treated 41 cases (67.2%) with mycophenolate mofetil and low-dose prednisone combined therapy and 20 cases (32.8%) were treated with full-dose prednisone alone. RESULTS Short-term response was significantly different between 2 groups (χ²=4.371, P=0.037), while no significant difference was found in long-term prognosis (χ²=0.419, P=0.522) after follow-up. The ROC curve showed that the most appropriate cutoff value was 30.67 μg·h/ml for MPA-AUC and the area under the ROC curve was 0.731, with 85.2% sensitivity and 64.3% specificity. CONCLUSIONS Mycophenolate mofetil and low-dose prednisone combined therapy is a reasonable treatment choice which can promote the remission of proteinuria without increasing obvious adverse reactions in pediatric HSPN with nephrotic state and pathological classification less than grade IV. MPA-AUC more than 30 μg·h/ml was an appropriate value for MMF in the combined therapy with MMF and steroid for treating children with HSPN.