Objective There are few studies on children with primary focal segmental glomerulosclerosis (FSGS) treated with rituximab (RTX).This study aimed to analyze the side effects and safety of the single dose of RTX in 10 children with primary FSGS, and further provide reference for the RTX treatment of FSGS . Methods We retrospectively analysed the clinical data of 10 FSGS children who hospitalized in the department of pediatrics in Nanjing General Hospital of Nanjing Military Area Command from April 2014 to August 2017.24 hours urinary protein, serum albumin, the count of circulating CD20+B cells and the RTX adverse reactions were analyzed after the treatment of RTX . Results From the 10 ca-ses, 6 cases were steroid-resistant nephrotic syndrome (SRNS), and 4 cases were frequently relapsing /steroid dependent nephrotic syn -drome (FRNS/SDNS).After single dose of RTX treatment , 5 cases achieved complete remission (CR), 2 partial remission (PR), and 3 non-remission (NR).Among the 6 cases of SRNS, 2, 1, 3 ca-ses achieved CR, PR, NR respectively; Among the 4 cases of FRNS/SDNS, CR was achieved in 3 patients, PR was achieved in 1 pa-tient.3 months after RTX treatment, urinary protein decreased from [2.41 (0.89-6.82) g/24 h] to [0.43 (0.05-1.1) g/24h], ser-um albumin increased from [31.60 (13.00-38.22) g/L] to [38.30 (27.18-53.20) g/L] .Adverse reactions occurred in 1 case in-cluding fever, chills, and chest tightness.These adverse reactions relieved after the decreased of RTX infusion speed .One developed severe pneumonia and proteinuria one month after RTX treatment .There was no increase in the number of infections in other children and no abnormalities in the respiratory tract , digestive tract, and nervous system during follow-up. Conclusion RTX treatment of primary FSGS has high security and has no serious adverse reactions .It is one of the effective treatments for children with FSGS .