Patients with PFAPA have an excellent prognosis. In the initial study of 94 PFAPA patients the fevers resolved in about 5 years (or after about 60 episodes) in 40 % of patients (34 of 83) [4]. In a later study, PFAPA resolved spontaneously in about 3 years [6]. Both medical and surgical options for management of PFAPA patients have been reported. Over 90 % of patients with PFAPA will dramatically become afebrile after 1 mg/kg dose of prednisolone. In a study of 105 patients, 74 patients used prednisolone for at least one PFAPA episode. Fifty-eight had their fevers resolve with one dose, 13 needed two doses, and one needed three doses spaced 12 h apart. Half the patients given prednisolone had the interval between episodes initially shorten by 7–14 days. However, over time of using prednisone the interval between febrile episodes returned to the baseline interval. Prednisolone (or prednisone) effectively aborts individual episodes of PFAPA but does not change the long-term outcomes of PFAPA. Since there are no laboratory tests available to establish the diagnosis of PFAPA, a dramatic resolution of fever with one or two doses of prednisolone is strong evidence that PFAPA is the diagnosis. In our personal experience (unpublished data), with febrile non-specific viral infections and asthma exacerbations, fever may persist following the first few doses of steroids. The use of prednisolone or prednisone relieves the fever and pharyngitis (but usually not the aphthous ulcers and adenitis) usually in a matter of hours. This response may be useful in distinguishing attacks of PFAPA from familial Mediterranean fever (FMF) or other hereditary autoinflammatory periodic fever syndromes.