Autoinflammatory syndromes include an expanding list of diseases characterized by unprovoked recurrent attacks of systemic inflammation with lack of autoantibodies or autoreactive T-cells. This group of conditions encompasses monogenic diseases with Mendelian inheritance which are caused by specific mutations of different genes regulating the innate immunity: familial Mediterranean fever, mevalonate kinase deficiency syndrome, tumor necrosis factor receptor-associated periodic syndrome, cryopyrin-associated periodic syndromes, pyogenic disorders and deficiency of interleukin-1 receptor antagonist: all these diseases can present with dermatological manifestations, which often represent the prominent clinical features or, in some cases, the presenting sign. The purpose of this review is to increase the recognition among clinicians and mostly dermatologists of the monogenic autoinflammatory syndromes, highlighting the cutaneous signs of these conditions, in consideration of the possibility to prevent irreversible damages when their diagnosis and treatment are precociously established.