The aim of the study was to study the characteristics of systemic lupus erythematosus (SLE) in the Egyptian population, comparing it to other populations. We retrospectively studied 207 patients with SLE diagnosed between 1990 and 2005. We obtained clinical features and laboratory data and analyzed them statistically. We studied 151 female and 56 male SLE patients. The female to male ratio was 2.7 to 1 and the mean age at presentation was 10 +/- 2.7 years (range 2-16). The mean disease duration was 6.47 +/- 3.74 years. At diagnosis, musculoskeletal, constitutional and mucocutaneous manifestations were the commonest features. During follow-up, the prevalence of nephritis (67%), hematological manifestations (44.9%), photosensitivity (44%), arthritis (39%), malar rash (38.2%), serositis (32.9%) and neuropsychiatric manifestations (24.25%) increased significantly. Those whose age of onset of the disease was <or=5 years (nine patients) had significantly more common hematological affection (P value = 0.0005). The characteristics of SLE in Egyptian patients show some similarities to other series of Middle Eastern countries, but with a lower female to male ratio. Disease onset below 5 years is extremely rare (4.35%), commonly presenting with hematological manifestations. The kidney was the commonest major internal organ involved, and also an important cause of death.