Systemic lupus erythematosus is a challenging disease with varied manifestations resulting from widespread immune complex deposition. It may present in the general pediatric office as an acute illness with fever, rash, and hematuria, or as chronic fatigue and malaise that might be mistaken for "school phobia". Although SLE remains an infrequent disease in general pediatric practice, mild cases are more frequent than was previously recognized. These children benefit from therapy if the physician is alert to their occurrence and makes the appropriate diagnosis. The understanding of the role of genetics and environmental agents in the pathogenesis of SLE has improved over the past 10 years. In addition, the past 10 years have seen refinements in the use of immunosuppressive regimens, such as cyclophosphamide, that have led to both improved quality of life and improved survival for children with active SLE unresponsive to corticosteroids. Although long-term concerns regarding the safety and efficacy of immunosuppressive drug regimens persist, the future is increasingly bright for children with SLE who receive appropriate care in a timely fashion.