The aim of the present study is to clarify the clinicopathological correlation of childhood IgA glomerulonephritis (GN) presenting diffuse endocapillary proliferation. Twenty-seven patients were used in the present study. The 27 patients were divided into three groups (mild, moderate and severe) according to the percentage of glomeruli displaying global endocapillary proliferation per total glomeruli at the first biopsy. The degree of both cellular crescent and lysis of the glomerular basement membrane (GBM) at the first biopsy was semiquantitatively evaluated. The degree of cellular crescent and lysis in the GBM was greater in the severe group than in the mild group. The degree of lysis of the GBM positively correlated with the degree of distribution of endocapillary proliferation. The degree of glomerular sclerosis at the second biopsy was greater in the severe group compared with the other two groups. The severity of cellular crescent at the first biopsy positively correlated with that of glomerular sclerosis at the second biopsy. Multiple logistic regression analysis indicated that the risk of glomerular sclerosis at the second biopsy was 19-fold higher in the odds ratio in the severe group compared with the mild group. In conclusion, the progression of glomerular sclerosis in serial biopsy is dependent on the degree of distribution of endocapillary proliferation and the severity of cellular crescents at the first biopsy in childhood IgA GN presenting diffuse endocapillary proliferation.