The cryopyrin‐associated periodic syndromes (CAPS) comprise three rare autoinflammatory disorders: familial cold autoinflammatory syndrome, Muckle–Wells syndrome, and neonatal‐onset multisystem inflammatory disorder. A mutation in the NLRP3 (CIAS1) gene, encoding cryopyrin, leads to inflammation and results in fever, chills, urticarial rash, arthralgias, conjunctivitis, and other difficult‐to‐control systemic features. Major advances in understanding interleukin‐1 (IL‐1) have led to better treatment options. This review focuses on a promising new medication, canakinumab, which is a recombinant, human anti‐human‐IL‐1β monoclonal antibody that has been effective in treating adult and pediatric CAPS. Drug Dev Res 72:553–560, 2011. © 2011 Wiley Periodicals, Inc.