Systemic lupus erythematosus (SLE) is an immune complex disease with many different clinical presentations. Here we report a 13-year-old female patient presenting with generalized lymphadenopathy, who meanwhile developed butterflly rash and pericarditis. The diagnosis of SLE was based on the clinical features, positive antinuclear antibody, and positive antibodies to dsDNA. The patient had an active disease and developed renal involvement, despite steroid therapy. The patient's clinical presentation, course and response to therapy are detailed, and the literature on lupus lymphadenitis is reviewed.