The autoinflammatory syndromes are group monogenic diseases related to mutations of genes involved in the control and in the regulation of the inflammatory response. All of them display an early onset in childhood. Familial Mediterranean Fever, Mevalonate-kinase deficiency and Tumour necrosis factor (TNF) Receptor-Associated Syndrome are characterised by recurrent episodes of systemic inflammation presenting as fever associated with a number of clinical manifestations, such as rash, serositis, lymphadenopathy, arthritis (also known as Periodic fevers). The mutation of the gene Cryopyrin is responsible of a spectrum of diseases (Familiary Cold Autoinflammatory Syndrome, Muckle-Wells Syndrome, and Chronic Infantile Neurological Cutaneous and Articular Syndrome) characterised by the dysregulation of IL-1 production and secretion. These disorders are characterised by a chronic or recurrent inflammatory condition variably associated with a number of clinical features, such as urticarial-like rash, arthritis, sensorineural deafness, central nervous system and bone involvement. Other diseases, such as Blau syndrome and Pyogenic Sterile Arthritis, Pyoderma Gangrenosum and Acne syndrome (PAPA) are characterised by a prevalent localisation of inflammation to specific organs and tissues, such as joints, skin and eyes. In the present review we will focus on the clinical presentation of these disorders in childhood and report on the available therapeutic strategies.