While immunodeficiencies, such as (Immunoglobulin A) IgA deficiency, may predispose pediatric patients to respiratory illnesses, they are also associated with autoinflammatory conditions. Distinguishing between these possible complications requires an awareness of these uncommon conditions. We report a case of a four-year-old boy with a history of IgA deficiency presenting to his primary care provider with fever of one week’s duration accompanied by abdominal, ear, and throat pain. The patient’s mother reported that he had these symptoms twice a month for the prior ten months during which he occasionally developed oral ulcers. Between episodes, the patient fully recovered without complications. Considering the patient’s history of IgA deficiency, it was originally assumed that the patient had strep throat and coincidental resolution of his symptoms after initiation of antibiotic therapy supported this diagnosis. However, due to the recurrent nature of his symptoms, a preliminary diagnosis of periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) was made. A referral to an otolaryngologist was made, and findings of adenopathy and tonsillitis suggestive of PFAPA were confirmed. The patient was scheduled for adenoidectomy and tonsillectomy, which were performed without complication, leading to the cessation of these episodes.
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