To study the demographic, clinical, laboratory features, treatment and outcome of childhood systemic lupus erythematosus (SLE) in western province of Saudi Arabia. Children with SLE who were diagnosed at King Abdulaziz University Hospital, Jeddah, between March 1998 and October 2008 were included. Charts of all patients were reviewed retrospectively for clinical and laboratory features, treatment and outcome. There were 28 girls and 2 boys, with a mean age of 10.5 years (range 5-18). The female:male ratio was 14:1. Constitutional symptoms represent significant symptoms. Hematological manifestations were the most frequent finding (86.7%) at the time of diagnosis followed by arthritis and nephritis (73.3%). The malar rash represents the most common skin manifestation (46.7%). Discoid lupus was very rare. Neurological symptoms were seen in 30%, while cardiac and pulmonary involvement was uncommon. All patients had positive ANA and 90% of them had high anti-ds DNA. All patients were treated with steroids and hydroxychloroquine and 26 patients received immunosuppressive therapy. Three patients died due to severe infection; massive brain infraction and severe lung disease (one in each). Twenty-seven patients are alive in stable condition. Clinical manifestations and laboratory abnormalities were similar to previously reported series. This report confirms that SLE has comparable findings among children from different ethnicities.