Objective:To investigate the composition of the renal disease spectrum and epidemiological characterisics for renal biopsy cases in Xinjiang Uygur Autonomous Region.Methods:The clinical and pathological data of 10 684 renal biopsy cases from 12 hospitals in Xinjiang Uygur Autonomous Region from August 1986 to December 2019 were collected and the composition of renal diseases and pathological types were analyzed retrospectively.Results:Among the 10 684 renal biopsy cases with 5 595 males and 5 089 females, 7 804 cases (73.04%) were Han nationality, 2 357 cases (22.06%) were Uygur nationality and 523 cases (4.90%) were other nationalities. Among the 10 684 cases of renal biopsy, primary glomerular disease, secondary glomerular disease, tubulointerstitial disease, end-stage renal disease, genetic and congenital disease and post transplant glomerular disease were 8 533 cases (79.87%), 1 740 cases (16.29%), 229 cases (2.14%), 121 cases (1.13%), 46 cases (0.43%) and 15 cases (0.14%), respectively. The distribution of kidney diseases in Han, Uygur and other nationalities (except Han and Uygur in this region) was the same as that in general. There was no significant difference in disease type composition between Han and Uygur, Han and other nationalities, and Uygur and other nationalities (all P>0.05). Among the 8 533 cases of primary glomerular diseases, the top five pathological types were IgA nephropathy (3 095 cases, 36.27%), mesangial proliferative glomerulonephritis (2 008 cases, 23.53%), membranous nephropathy (1 503 cases, 17.61%), minimal glomerulopathy (567 cases, 6.64%) and focal segmental glomerulosclerosis (494 cases, 5.79%). The top five pathological types of primary glomerular diseases were different between Han and Uygur, and Han and other nationalities (both P<0.01). There was no statistically significant difference between Uygur and other nationalities in the top five pathological types of primary glomerular diseases ( P=0.113). Among 1 740 cases of secondary glomerular diseases, the top five pathological types were lupus nephritis (517 cases, 29.71%), Henoch-Sch?nlein purpura nephritis (304 cases, 17.47%), diabetic glomerulosclerosis (285 cases, 16.38%), benign renal arteriosclerosis (196 cases, 11.26%) and systemic vasculitis (101 cases, 5.80%). It was different between Han and Uygur, Han and other nationalities, and Uygur and other nationalities in the top five pathological types of secondary glomerular diseases. Conclusions:Primary glomerular disease accounts for 79.87% of renal diseases in Xinjiang Uygur Autonomous Region. IgA nephropathy is the main pathological type, followed by mesangial proliferative glomerulonephritis and membranous nephropathy. The most common pathological type of secondary glomerular disease in this region is lupus nephritis, followed by Henoch-Sch?nlein purpura nephritis and diabetic glomerulosclerosis. The top five pathological types of primary glomerular diseases and secondary glomerular diseases are different in different ethnic groups in Xinjiang Uygur Autonomous Region.