Familial Mediterranean fever (FMF) is the most common monogenic autoinflammatory disease worldwide and a key-model to illustrate dysregulation of innate immunity, etiologically determined by pathogenic variants in the <i>MEFV</i> gene, encoding pyrin, leading to uncontrolled interleukin-1β and interleukin-18 release. This evidence concerns the gene IL18 and familial Mediterranean fever.