Cryopyrin-associated periodic syndrome (CAPS) is an autoinflammatory disorder caused by heterozygous mutations in the NLRP3 gene and include three distinct conditions, namely familial cold autoinflammatory syndrome, Muckle-Wells syndrome, and chronic infantile neurologic, cutaneous, and articular (also known as neonatal-onset multisystem inflammatory disease) (chronic infantile neurologic, cutaneous, and articular/neonatal-onset multisystem inflammatory disease (CINCA/NOMID)) syndrome [1–5]. Here, NLRP3 is linked to CINCA syndrome.