The autoinflammatory syndromes are a group of multisystem disorders characterized by recurrent episodes of fever and systemic inflammation affecting the eyes, joints, skin, and serosal surfaces in the absence of an immune reaction. Recent advances have revealed the importance of interleukin-1β, not only in the pathogenesis of many of these rare inherited diseases, but also in acquired diseases. The development and availability of anti-interleukin-1β therapeutics have introduced the possibility of proof-of-concept studies, which are likely to further widen this field.