Recurrent autoinflammatory fever syndromes are characterized by an abnormal activation of the innate immune system pathways, leading to inappropriate systemic inflammation responsible for clinical symptoms. The diagnosis of these conditions is difficult because of their low prevalence, but also because of their nonspecific clinical signs. The presence of clinical inflammatory signs such as fever associated to serositis (arthritis, peritonitis …) or recurrent cutaneous manifestations and elevated acute phase reactants should help the clinician to make the correct diagnosis. The purpose of this article is to provide a practical management flow chart for children with a suspected autoinflammatory recurrent fever syndrome.