INTRODUCTION Systemic lupus erythematosus (SLE) is an unpredictable autoimmune systemic disorder that often involves the kidney. In this study, we aimed to assess the clinical characteristics, pathological findings, and therapeutic response of children presenting with lupus nephritis (LN). METHODS We retrospectively studied 50 children with SLE admitted to the pediatric nephrology departments of four teaching hospitals in Baghdad between December 2009 and December 2011. RESULTS The female to male ratio was 5:3. The median age was 11 years with a range of 5 -17 years. The commonest presenting features were weight loss (92%), fever (90%), edema (88%), arthritis (70%), hair loss (70%) and malar rash (60%). Examination revealed hepatosplenomegaly/lymphadenopathy in 20% of patients. Anti-dsDNA was positive in 92% of patients. Pathological examination revealed LN class I in 4%, class II (10%), class III (8%), class IV (74%) and class V (4%). All patients in class I, II and III achieved full remission after treatment with prednisolone. Thirty-one patients in class IV and V received induction treatment with cyclophosphamide followed by maintenance with cyclophosphamide (16 patients), mycophenolatemofetil (13 patients) or azathioprine (2 patients). Twenty-four of those patients achieved remission, four patients developed chronic kidney disease and three patients died. Five patients in class IV/V were treated with mycophenolate mofetil and three of them achieved remission, whilst three patients were treated with azathioprine and all of them achieved remission. CONCLUSION Diffuse proliferative glomerulonephritis (class IV) was the most common pattern of LN encountered in our study.