OBJECTIVE To investigate the clinicopathologic characteristics of childhood renal diseases.METHODS A retrospective analysis of 1316 renal biopsies performed over the past 20 years was performed. RESULTS Of the 1316 patients, 383(29.09% ) were diagnosed as nephrotic syndrome, 291(22.00%) as acute nephritis syndrome, 224 (17.21%) as isolated hematuria, 209 (15.87%) as purpura nephritis, and 96(7.30% ) as hepatitis B virus-associated nephritis . Mesangial proliferation was the most common pathological change (756 cases; 57.45%), followed by IgA nephropathy (113 cases; 8.59%), endothelial capillary proliferation(112 cases; 8.51%), membranous nephropathy (66 cases; 5.02%), and various minor and minimal changes (59 cases; 4.48%). Alport syndrome, congenital nephrotic syndrome, thin basement membrane nephropathy, fibrillary glomerulopathy disease, and Fabry disease were confirmed by electronic microscopy. IgA, IgM and C1q nephropathy were definitely diagnosed using immune histochemistry or immunofluorescent. A diagnosis of primary glomerular disease was made in 69.53% of the cases (915 cases); secondary glomerular disease was noted in 26.14%(344 cases). Of the 915 cases of primary glomerular disease, 375 (941.0%) had nephrotic syndrome. Secondary glomerular disease due to purura nephritis was common (209/344; 60.8%). CONCLUSIONS Primiary glomerular disease predominates in children. Nephrotic syndrome is the most common clinical diagnosis. Mesangial proliferation is the most common pathological patterns in children with renal disease.［Chin J Contemp Pediatr, 2007, 9 (2) :117-121］