A subverted inflammasome homeostasis leading to IL-1 oversecretion characterizes the cryopyrin-associated periodic syndrome (CAPS), with three clinical sceneries of increasing severity which usually start in the first infancy [9]: familial cold-induced autoinflammatory syndrome (FCAS), Muckle-Wells syndrome (MWS) and chronic infantile neurologic cutaneous articular syndrome (or CINCA syndrome, also referred as “neonatal onset multi-system inflammatory disorder”), all caused by dominant missense mutations in the NACHT domain of the same NLRP3 gene encoding the cryopyrin protein. The gene discussed is NLRP3; the disease is cryopyrin-associated periodic syndrome.