Hereditary fevers are a group of rare disorders of the inflammatory response. Clinical features include recurrent attacks of fever and organ-localized inflammation. Minimal clinical variations, a unique biochemical-specific abnormality, and the mode of genetic inheritance distinguish the three main diseases: familial Mediterranean fever, hyperimmunoglobulinemia D and periodic fever syndrome, and autosomal dominant recurrent fever. The complete elucidation of pathogenesis of these intriguing disorders will be provided by the genetic studies currently in progress.