To provide an overview of the epidemiology of dietary protein-induced enterocolitis syndrome (FPIES). FPIES is a very uncommon non-IgE-mediated gastrointestinal allergy disease. Older non-population-based research reported an average of 1–15 cases presenting to allergy clinics each year, whereas more recent studies have found as many as 90 cases per year. In one research, the annual incidence of FPIES was one in 10,000 infants under the age of two. Chronic FPIES mainly affects newborns, but acute FPIES predominantly affects young babies. FPIES is more frequent in men; eczema and a family history of atopy are common at diagnosis; almost one in ten babies has to coexist IgE food allergies, and siblings are seldom impacted. Regional differences exist in common triggering foods, rates of combined cow milk and soy FPIES, and rates of multiple food groups FPIES. The lack of a broadly recognized definition and the publishing of a few prospective population-based case series restrict our understanding of the epidemiology of FPIES.

FPIES is not as uncommon as previously assumed, but future well-designed population-based studies must examine how frequent it is, what variables predispose to its development, and why there is regional heterogeneity.