Mutations in the NLRP3 gene (formerly known as CIAS1) have been shown to cause a spectrum of autoinflammatory diseases including familial cold autoinflammatory syndrome (FCAS), Muckle-Wells syndrome (MWS), and neonatal-onset multisystem inflammatory disease (NOMID)/chronic infantile neurologic, cutaneous, and articular syndrome (CINCA) [1]. This evidence concerns the gene NLRP3 and CINCA syndrome.