Abstract: 　Objectives　To investigate the composition of clinical classification and pathological patterns and their relationships and change in children with renal disease undergoing biopsy. Methods　A retrospective analysis of pathological and clinical data obtained from children ( ≤ 14 year) with renal disease undergoing biopsy from 1984-1997 and from 1998-2011 was performed. Results　One thousand four hundred and sixty-two children underwent renal biopsy in 28 years, and 1313 patients were recruited in this study, 824 males (62.8%) and 489 females (37.2%). The mean age was 9 years and 4 months at renal biopsy. There were 921 children (70.1%) with primary glomerular disease (PGD) and 312 children (23.8%) with secondary glomerular disease (SDG). The main clinical classifications of PGD were nephrotic syndrome (NS, 31.2%), isolated hematuria (IH, 16.1%), and acute glomerulonephritis (AGN, 11.0%). The main pathological patterns of PGD were IgA nephropathy (IgAN, 27.6%), minimal change disease (MCD, 24.0%), and mesangial proliferative glomerulonephritis (MsPGN, 16.9%). The main causes of SGD were lupus nephritis (LN, 40.7%), Henoch-Sch?nlein purpura nephritis (HSPN, 34.3%), and hepatitis B virus related glomerulonephritis (HBV-GN, 19.6%). In this 28 years, the composition of PGD was decreased, however, the compositions of SGD and other renal diseases were increased. Compared with 1984-1997, the pathological manifestations of IgAN, MCD and focal segmental glomeralosclerosis were increased, MsPGN, IgMN, and crescentic glomerulonephritis were decreased in 1998-2011. The difference was statistically significant (P<0.05). In SGD patients, HBV-GN was significantly decreased (P<0.05). Conclusions　PGD is the main disease in children undergoing renal biopsy. IgAN is the most common pathological pattern. NS is the most common clinical classification. In this 28 years, the composition of PGD is decreased, SGD and other renal diseases are increased in children undergoing renal biopsy.