Objective To explore the clinical characteristics of children with lupus nephritis (LN) complicated with neuropsychiatric systemic lupus erythematosus (NPSLE), and to analyze the risk factors of LN complicated with NPSLE.Methods A total of 119 children with LN who were admitted to the Department of Nephrology, Xi'an Children's Hospital from May 2017 to January 2024 were enrolled. They were divided into NPSLE group (n=26) and non-NPSLE group (n=93) according to the presence or absence of NPSLE. Clinical data were compared between the two groups, and the clinical characteristics and risk factors of LN children with NPSLE were analyzed.Results The incidence of NPSLE in children with LN was 21.8%, with male-to-female ratio of 1∶5.5, and an average age of 10 years and 6 months. The most common neurological manifestations were psychiatric abnormalities, erythema discoideum in general, and abnormalities of cranial MRI on imaging. There were significant differences in antinuclear antibody positivity, anti-ds-DNA antibody positivity, anti-Smith (anti-SM) antibody positivity, reduction of white blood cells, red blood cells and platelets, performance of only anemia, high erythrocyte sedimentation rate (>15 mm/h), hypoproteinemia, positive Coombs test, hematuria and massive proteinuria between the two groups (P<0.05). Binary logistic regression analysis showed that hematuria, massive proteinuria and positive anti-SM antibody were independent risk factors for NPSLE in children with LN. Conclusion A variety of factors can influence whether LN is comorbid with NPSLE, with children with LN who have haematuria, large amounts of proteinuria, and positive anti-SM antibodies being prone to comorbid neuropsychiatric changes.