Subcutaneous canakinumab (Ilaris®) is a human monoclonal anti-human interleukin (IL)-1β antibody of the immunoglobulin G1/κ isotype that binds with high affinity and specificity to human IL-1β, blocking its interaction with IL-1 receptors. It is approved in the EU as monotherapy or in combination with methotrexate for the treatment of patients aged ≥2 years with active systemic juvenile idiopathic arthritis (SJIA) who have responded inadequately to previous therapy with non-steroidal anti-inflammatory drugs and systemic corticosteroids. In the USA, it is indicated for the treatment of patients aged ≥2 years with active SJIA. In two placebo-controlled, multinational, phase III studies in patients aged 2–19 years with SJIA, canakinumab rapidly reduced disease activity, permitted the tapering of glucocorticoid therapy and delayed the time to disease flare. The efficacy of canakinumab was sustained at a median follow-up of 49 weeks in an ongoing extension study. In clinical studies, canakinumab had an acceptable tolerability profile that was comparable with that observed in patients with cryopyrin-associated periodic syndromes. In general, adverse events were mild or moderate in intensity, with nasopharyngitis, cough, pyrexia, vomiting, diarrhoea and upper respiratory tract infection the most frequently reported treatment-emergent adverse events. Thus, current evidence suggests subcutaneous canakinumab extends the treatment options currently available for patients aged ≥2 years with SJIA.