Cryopyrin-associated periodic syndromes (CAPS) are a group of rare, monogenic, autoinflammatory diseases caused by a gain of function mutation in NLR family pyrin domain containing 3 (NLRP3), which results in excess production of interleukin-1 beta (IL-1β) [1]. The gene discussed is NLRP3; the disease is cryopyrin-associated periodic syndrome.