Abstract: Childhood-onset systemic lupus erythematosus (cSLE), which refers to SLE before the age of 18, is the second systemic connective tissue disease in children. Compared with adults, cSLE has its own characteristics in epidemiology, clinical presentation, and treatment. In recent years, with the rapid development of human genomics and molecular biology, the understanding of the disease has been updated. This paper reviewed recent progress in the etiology, manifestation, and treatment of cSLE, so as to better understand this disease and guide clinical practice.