Lupus nephritis is a common manifestation of systemic lupus erythematosus and a strong predictor of poor outcome. LN is more frequent in children than in adults. Overall, 60-80% of children with SLE have urinary or renal function abnormalities early in the disease course. Despite decades of research, the precise immuno-pathogenesis of LN has remained elusive.Clinically significant renal involvement ranges from asymptomatic urinary findings to nephrotic syndrome and renal failure.Diagnosis of LN depends on clinical picture and laboratory investigations which demonstrate autoantibodies especially anti ds DNA. Recently, it found that urinary monuclear cells from children with LN were the clearly dominant cell type. New therapies for LN have to be at least as effective as and less toxic than existing therapies. Novel immunological targeted therapies include both B and T cell directed therapies, anti-cytokine therapies, complement directed treatments as well as immunoablative chemotherapy with heomopoietic stem cell rescue.