Lupus nephritis (LN) is one of the major clinical manifestations of systemic lupus erythematosus (SLE) which occurs frequently in the early stages of pediatric-onset cases. Since SLE is a chronic disease associated with frequent disease flares and effective and safe maintenance therapy is required for achieving a favorable outcome, optimal treatment for LN in pubertal patients is a great challenge that remains to be overcome. Although its etiology remains unclear, it has been reported that the innate and adaptive immune systems have been reported to play an important role in the pathogenesis of SLE. However, studies of drugs that have been useful in controlling inflammatory pathways mediated by the innate and adaptive immune systems are now underway. In clinical practice, recent advances in the management of LN, together with earlier renal biopsy and selective use of aggressive immunosuppressive therapy, have contributed to a favorable outcome in children and adolescents with LN. However, the balance of the efficacy of treatment in terms of long-term prognosis and its adverse effects should be weighed in determining the treatment strategy.