Food protein‐induced enterocolitis syndrome (FPIES) is a non‐IgE-mediated food allergy that primarily affects the gastrointestinal tract. The underlying pathophysiology of FPIES has yet to be fully elucidated; however, FPIES is believed to be secondary to intestinal inflammation after exposure to a food antigen, which thereby leads to increased permeability and fluid shifting into the intestinal lumen. FPIES is categorized into acute and chronic forms. Acute FPIES is characterized by repetitive vomiting that occurs 1‐4 hours after food ingestion. Severe vomiting may progress to dehydration, lethargy, and pallor, which potentially leads to hypovolemic shock. In some patients, diarrhea may present within 24-hours of food ingestion. Patients are clinically well between acute episodes. Chronic FPIES presents with intermittent vomiting and/or diarrhea, followed by failure to thrive. FPIES characteristically presents in infancy, with resolution of the disease typically occurring by school age. However, analysis of recent data indicates that FPIES may persist into adulthood. In addition, late- or adult-onset FPIES has also been reported. The diagnosis of FPIES is based on clinical history; however, oral food challenge currently remains the criterion standard for diagnosis. Management of FPIES requires strict avoidance of food triggers, and treatment requires rapid fluid rehydration. Currently, there are no reliable biomarkers to diagnose FPIES; however, investigations to better understand the role of the innate immune system have been promising. Future studies are needed to better understand the true prevalence and pathophysiology of FPIES.