Neutrophils, increasing evidence suggests, play an important part in the development and perpetuation of autoimmunity in patients with systemic lupus erythematosus (SLE). In this Review, the author highlights defects in the phenotype and function of SLE-derived neutrophils. Intriguing links between aberrant neutrophil death, production of proinflammatory mediators, and the presentation of and response to autoantigens are explored. The potential contribution of defective neutrophil activity to organ dysfunction is also discussed.