Introduction: Gain-of-function mutations in the NLRP3 gene, lead to hyperactivation of the NLRP3 inflammasome, resulting in the inappropriate release of inflammatory cytokines including IL-1β, and cause a spectrum of autosomal-dominant systemic autoinflammatory diseases called cryopyrin-associated periodic syndromes (CAPS). The gene discussed is NLRP3; the disease is cryopyrin-associated periodic syndrome.