Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis syndrome (PFAPA) was first described in 1987[
1]. The diagnosis of PFAPA is clinical, requiring the presence of characteristic features and the exclusion of alternative causes. Accepted diagnostic criteria include [
1] regularly occurring fevers with an early age of onset [
2] symptoms in the absence of upper respiratory tract infection with at least one of the following clinical signs: aphthous stomatitis, cervical lymphadenitis, and pharyngitis, [
3] exclusion of cyclic neutropenia, [
4] complete absence of symptoms between episodes, and [
5] normal growth and development [
2]. Updated clinical classification criteria were established in 2019 requiring patients to fulfill at least seven out of the following 8 manifestations including the presence of pharyngitis, 3–6 day duration of episodes, cervical adenitis, and periodicity as well as the absence of diarrhea, chest pain, skin rash and arthritis [
3]. The etiology of PFAPA syndrome remains unclear, though it is generally thought to be the result of immune dysregulation. It is considered a non-hereditary disease; however, this has been the subject of debate in recent literature [
4]. PFAPA syndrome is self-limited with a favorable prognosis. Symptomatic treatment can be achieved with oral glucocorticoids, which result in dramatic resolution of fever, but do not prevent recurrence. In most children, fever episodes become less frequent over time and eventually cease altogether. Tonsillectomy is an effective option for decreasing the frequency and severity of episodes with a significant potential for cure [
5].