The objective was to present a case of periodic fever with aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA), summarize the medical literature on PFAPA, review the differential diagnosis and suggest a diagnostic approach to periodic fevers in children. A PubMed search was conducted for all case reports and series of patients with PFAPA. The references of these papers yielded further case reports. Review articles or large case series were used for sources of information regarding the other periodic fever and autoinflammatory syndromes. All cases reported as PFAPA were included in the review, even though a few of the cases may not have been accurately diagnosed. The periodic fever and autoinflammatory syndromes of childhood are a group of diseases that cause repeated febrile illnesses with various associated symptoms. Except for PFAPA, each of these diseases is caused by a known genetic mutation. Effective treatment options and long-term prognosis varies among these syndromes. Children with periodic fever or autoinflammatory syndromes sometimes present to an Allergy/Immunology clinic for immunologic evaluation. It is important for the Allergy/Immunology specialist to be familiar with the clinical presentation, diagnostic approach and treatment of these conditions.