Abstract Systemic lupus erythematosus (SLE) is the prototypic autoimmune disorder, characterised by auto-antibody production against nuclear antigens. Juvenile-onset systemic lupus erythematosus (JSLE) is a more severe form than the adult-onset disease, with a more balanced gender ratio. The disorder is of unknown aetiology with hormonal, genetic and environmental factors all thought to play a role. Diagnosis is notoriously difficult due to the protean clinical signs and symptoms. Specialist input is necessary given the severity and range of clinical manifestations during an important time of growth and development. A personalized approach is required to provide optimal care for an individual's needs. A holistic, multi-disciplinary approach to the management of JSLE is crucial. Medical therapy aims at dampening down the immune response. Biological therapies that target specific points in the immune system are becoming available. Clinical trials in JSLE are sparse with much of the management guided by evidence derived from adult-onset SLE. The first trial of biologic use in JSLE is currently underway. Further investigations of aetiology and clinical trials in JSLE are required to inform management and identify targets for future therapy.