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Systemic lupus erythematosus in children is uncommon, but not rare. It should be considered in any child (particularly an older child) who has been unwell for more than 1 week with multisystem clinical features without a diagnosis. The most common presentation is a febrile child with fatigue, an erythematosus malar rash and arthritis. Leukopenia, thrombocytopenia and a raised ESR is suggestive, as is a urinalysis indicative of nephritis. The presence of anti-DNA antibodies confirms the diagnosis, but its absence does not exclude this possibility. Although a life-long and serious disease with a high morbidity and substantial mortality, the prognosis has dramatically improved recently, largely because of the aggressive use of high-dose corticosteroids at onset and with disease flares, combined with other potent anti-inflammatory agents. The early empirical use of antibiotics for possible sepsis is essential. Early referral and frequent follow-up by a team specialized in the management of the condition has probably been critical in improving outcome.