OBJECTIVE: To investigate the clinicalpathologic characteristics of IgM nephropathy in children. METHODS: The data of 34 children with IgM nephropathy from the First Affiliated Hospital of Zhengzhou University were retrospectively reviewed. RESULTS: Of the 34 cases of IgM nephropathy, nephrotic syndrome (NS) was clinically presented in 22 cases (64.7%). The renal pathological classification was as follows: minimal change disease (12 cases, 35.3%), minimal change disease with acute renal tubular injury (3 cases, 8.8%), minimal change glomerulonephritis (6 cases, 17.6%), minimal change glomerulernephritis with ischemic renal injury (1 case, 2.9%), mesangial proliferative glomerulonephritis (7 cases, 20.6%), focal segmental glomerulosclerosis (4 cases, 11.8%), focal proliferative glomerulernephritis (1 case, 2.9 %). Glomerular injury score, renal vascular injury score and total renal injury score increased with the increasing IgM deposition. CONCLUSIONS: The majority of children with IgM nephropathy manifest clinically as nephrotic syndrome. The patterns of renal pathology may be varied in children with IgM nephropathy. IgM deposition in the mesenteric area is an important pathologic feature and is related to the degree of renal injury.［Chin J Contemp Pediatr, 2010, 12 (5):338-340］