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The exact pathogenesis and the aetiology of <jats:styled-content style=\"fixed-case\">PFAPA<\/jats:styled-content> are still unknown.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>We conducted a non\u2010systematic review of published articles about <jats:styled-content style=\"fixed-case\">PFAPA<\/jats:styled-content> syndrome and summarised the evidence for diagnostic criteria and treatment options for <jats:styled-content style=\"fixed-case\">PFAPA<\/jats:styled-content>.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>The first proposed diagnostic criteria for <jats:styled-content style=\"fixed-case\">PFAPA<\/jats:styled-content>, in addition to periodic fever, included aphthous stomatitis, pharyngitis or cervical lymphadenitis in children younger than five years at the beginning of the symptoms. C\u2010reactive protein (<jats:styled-content style=\"fixed-case\">CRP<\/jats:styled-content>) levels and leucocyte counts increase in most patients during episodes. Recent research reveals that tonsillectomy provides an immediate and long\u2010lasting cure for <jats:styled-content style=\"fixed-case\">PFAPA<\/jats:styled-content>, even in the absence of classic criteria of aphthous stomatitis, pharyngitis or cervical adenitis and in children older than five years.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>We suggest that <jats:styled-content style=\"fixed-case\">PFAPA<\/jats:styled-content> can be diagnosed in children with at least five regularly occurring fever episodes without any other explanation, even in the absence of aphthous stomatitis, pharyngitis or cervical lymphadenitis and also in children older than five 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