To highlight the characteristics of Egyptian children with systemic lupus erythematosus (SLE), the records of 52 SLE patients (48 girls and four boys aged 11.9+/-2.6 years) were retrospectively analyzed. The median duration of follow up was 22 months (range 1-94.5). The most common extrarenal manifestation was fever (76.6%), followed by joint involvement (65.4%). Hemolytic anemia was demonstrated in 51%, thrombocytopenia in 29.2%, and leucopenia in 27.5%. Antinuclear antibodies were positive in 92.7%, while positive anti-double-stranded DNA and hypocomplementemia were demonstrated in 95.6% and 67.4%, respectively. Lupus nephritis (LN) was evident in 80.8%. The renal manifestations of LN patients were proteinuria (83.3%), hematuria (71.5%), hypertension (35.7%), and elevated serum creatinine (16.7%). The histopathological findings of the initial renal biopsies were class I (4.9%), class II (22%), class III (36.3%), and class IV (36.3%). Among patients without LN, 85.7% gained remission and nonimmediately died. At last observation, 55.6% of LN patients had complete remission, 22.2% had active disease, and 22.2% died. Most patients who died had class IV LN. In conclusion, the characteristics of Egyptian SLE children are comparable with those in most Arab and Western series. However, LN may be more prevalent and severe, with unfavorable outcomes.