Objective To analyze the clinic pathological features and long⁃term prognosis in lupus nephri⁃ tis（LN）children of different gender. Methods The clinical and pathological data on children with LN treated in the First Affiliated Hospital of Sun Yat⁃sen University from January 1，2003 to December 31，2019 were retrospectively analyzed. Results In the 343 children，the male to female ratio was 1∶4.04. Girls had a higher incidence of alope⁃ cia and photosensitivity than boys（P < 0.05），whereas boys showed a higher rate of thrombocytopenia and in⁃ creased serum creatinine（P = 0.042）. A total of 227 children，including 54 boys and 173 girls，underwent renal biopsy. There was no significant difference in pathological types between boys and girls（P = 0.089）. Leucocyte in⁃ filtration and nuclear fragmentation were more common in boys（P < 0.05）. There were no significant differences in the rates of remission and relapse after induction therapy and the long⁃term prognosis between boys and girls（P > 0.05）. Conclusions The clinic pathological features differed in LN children of different gender. Boys were prone to severer hematologic and kidney diseases. The effectiveness of induction treatment and the long⁃term prognosis were similar between boys and girls with LN.