Abstract: Systemic Lupus Erythematosus is not rare in Iran. We have seen 220 cases in children in a population of 893 SLE in the past 16 years. In this study we show the features of SLE in children. The female to male ratio was 86/14. Fever was present in 72% of the patients, weight loss in 40%, malar rash in 72.5%, photosensitivity in 54%, mucosal lesion in 48%, discoid rash in 13%, transient polyarthritis (less than 6 week) in 19%, proteinuria (>500 mg/24 hours) in 46%, and cylindruria in 34% of the patients. Kidney biopsy was performed in 43% of them. The light microscopy revealed: 71% WHO class IV and 22% class III glomerulonephritis: Pericarditis and pleurisy was seen in 26.5%, splenomegalia in 23%, Ieukopenia in 25%, lymphopenia in 37%, thrombocytopenia in 18%, hemolytic anemia in 5.9%, ESR > 50 mm Hg in the 1st hour in 73%, positive VDRL in 20%, positive FANA in 26%, positive LE cells in 63.3%, elevated anti DNA in 82%, low C3 in 59% and low C4 in 61% of the patients. Comparison of SLE in children with adult in Iran shows some differences. The features of SLE in Iran, both in adults and children, differ from western countries.