Genotype-phenotype correlation studies divided NLRP3 variants into three groups, depending on the associated clinical picture, which can be (i) at the ends of the severity spectrum continuum (i.e., FCAS and CINCA); (ii) contiguous CAPS phenotypes, such as FCAS/MWS or MWS/CINCA phenotypes; and (iii) related to polymorphisms or low-penetrance variants. This evidence concerns the gene NLRP3 and CINCA syndrome.