Abstract : Autoinflammatory diseases refer to a broad spectrum of diseases that are primarily due to an abnormality in the regulation of natural immunity. Some are polygenic and highly influenced by the environment, others are monogenic. This article is devoted to a family of monogenic autoinflammatory diseases that is very important because it includes the emblematic Mediterranean familial fever, the first autoinflammatory disease described as such and which heavily affects the Eastern Mediterranean populations. We will discuss the regulatory mechanisms of inflammasomes and the impact of certain mutations on their function. General principles of treatment and diagnosis will also be discussed. Other autoinflammatory diseases (including type 1 interferonopathies and NF-?b and TNF-? axis abnormalities) deserve to be discussed later on.