Juvenile-onset  systemic lupus erythematosus (SLE) is a significant and urgent problem in the practice of pediatricians and rheumatologists  in both verifying its diagnosis and choosing treatment policies, by taking into account the features of the onset, the types of the disease course, and the high probability of an unfavorable prognosis. Despite the tremendous progress made in rheumatology at the beginning of the 21st century, the results of SLE therapy in children and adolescents cannot be considered to be satisfactory: only 13% of patients who fell ill in childhood have drug-free remission in adulthood with a lower quality of life and a higher index of damage. The purpose of this review is to analyze the efficacy and safety of rituximab that, despite the absence of its officially recorded indications,  has relatively widely entered the actual clinical practice of pediatric rheumatologists  as the drug of choice in the highly active, prognostically unfavorable course of SLE.