Abstract: Objective To investigate the clinical and pathological features of Henoch-Schönlein purpura nephritis (HSPN) in children. Methods The clinical and pathological data of 242 children with HSPN admitted from January 2009 to January 2014 were retrospectively analyzed. Results The most common clinical type was proteinuria and hematuria (135 cases, 55.79%). The mainly pathological types were type Ⅱ and Ⅲ. The distribution of pathological types was statistically significant between different clinical types (P=0.000). The patients with normal urine test had the lowest pathological grade. The patients with isolated hematuria and proteinuria mainly had pathological type Ⅱ. The patients with hematuria and proteinuria mainly had pathological type Ⅲa. The patients with nephrotic syndrome mainly had pathological type Ⅲb. The length of the courses at renal biopsy was not statistically significant among different clinical types (P>0.05) and pathological types (P>0.05). The deposition of immune complex in kidney was not statistically significant among different pathological types (P>0.05). The levels of IgA, C3, and platelet count were not statistically significant among different clinical and pathological types (P>0.05). Conclusion The clinical classification is related to the pathological grade in children with HSPN.