Background: Despite several years of intensive investigation, relatively few studies have been made of children with lupus nephritis. The prognosis of children with lupus nephritis is poor for those with diffuse proliferative glomerulonephritis and active interstitial inflammation. As newer treatment modalities become available for patients with severe lupus nephritis, it become increasingly important to identify patients at risk for renal failure. The aim of this study was to evaluate the clinical course, histopathology, serologic features and prognostic significance of some parameters, to identify the risk factors for renal failure and mortality in children with lupus nephritis. Materials and Methods: Retrospectively 30 children under 16 years of age with lupus nephritis from 1989 to 1999 were studied. All patients received renal biopsy and follow-up biopsies were performed in 3 children. Lupus nephritis was classified by the World Health Organization (WHO) criteria. The clinical and serologic parameters at the time of renal biopsy were recorded.Results: All children underwent renal biopsy within 1 year of disease onset. There were 1 (3.3%) patients with class II, 5 (16.7%) with class III, 21 (%70) with class IV, and 3 (%10) with class V nephritis based on initial biopsy according to the WHO classification. The mean follow-up time was 60 months. Follow-up biopsies were histologically stationary in 2 patients and progressive in one. The overall renal and patient 5- year survival rates were 46.66% (14/30) and 93.33 %( 2/30) respectively. They were 47.61% (10/21) and 95.21 %( 20/21), respectively, of patients with class IV proliferative glomerulonephritis. Children with renal pathology (class V in the WHO classification system) at initial biopsy, were at high risk for renal failure 66.66% (2/3) or morality %33.33 (1/3) despite aggressive treatment. The results revealed that those with persistent hypertension, anemia, and decreased creatinine clearance rate, nephrotic proteinuria, at initial biopsy were more prone to develop renal failure (P<0.01).Conclusion: The prognosis of children with class IV nephritis in our study was better than reported in other series in recent years. However, those with class V disease, persistent hypertension, anemia, low creatinine clearance and nephrotic proteinuria at the time of diagnosis are at increased risk for renal failure. The improved results may be due to initial histological classification, better supportive care and selective use of aggressive therapy such as methylprednisolone pulse therapy and intravenous cyclophosphamide for those with high risk factors.