The study aimed to investigate the spectrum of biopsy-proven kidney disease in Chinese children. Records of children 0-17years old who underwent native kidney biopsy from June 1st, 2013 to December 31st, 2018 in the national inpatients' database of China were analyzed. Biopsy-proven kidney diseases of different sex, age groups, and diagnosis, and the changing patterns of kidney disease compared with the previous study were analyzed. A total of 21,311 patients from 232 hospitals with a median age of 11.34years were included. Immunoglobulin A vasculitis with nephritis (IgAVN) was the most common pathological finding [29.17%, 95% confidence interval (confidence interval, CI) = 28.56-29.78], followed by IgA nephropathy (IgAN) (22.70%, 95% CI = 22.14-23.27). IgAN was the most common finding in patients with hematuria (60.75%, 95% CI = 58.83-62.65], proteinuria (33.43%, 95% CI = 30.54-36.42), and hematuria plus proteinuria (62.77%, 95% CI = 56.19-69.02). Minimal change disease was the most common finding (40.69%, 95% CI = 39.41-41.98) in nephrotic syndrome. The proportion of IgAVN in patients with biopsy-proven glomerular disease increased year by year during 2013-2018 (p for trend < 0.001) and washigher than that of2004-2014 [29.41% (95% CI = 29.10-29.72) in 2013-2018 vs. 13.35% (95% CI = 12.97-13.73) 2004-2014, p < 0.001]. The proportion of hepatitis B virus associated nephritis during 2013-2018 was lower than that of 2004-2014 [0.44% (95% CI = 0.36-0.54) in 2013-2018 vs. 0.87% (95% CI = 0.67-1.10) in 2004-2014, p < 0.001]. IgAVN and IgAN were the most common types of pathological findings in children who underwent kidney biopsies from 2013 to 2018. The pathological spectrum of kidney biopsy changed over time.