Food Protein-Induced Enterocolitis Syndrome (FPIES) is a symptom complex of severe vomiting and diarrhea caused by non-IgE-mediated allergy to cow's milk and/or soy in infants. Symptoms typically begin in the first month of life in association with failure to thrive and may progress to acidemia and methemoglobinemia. Symptoms resolve after the causal protein (usually sensitivity to both cow's milk and soy) is removed from the diet. Symptoms recur approximately 2 hours after reintroduction of the protein along with a coincident elevation of the peripheral blood polymorphonuclear leukocyte count. The sensitivity is usually outgrown by 3 years of age. The purpose of this review is to delineate the characteristic clinical features, diagnosis and management of FPIES. Furthermore, infantile FPIES will be discussed in relation to clinical syndromes that share features with it ("atypical FPIES") and other food-allergic disorders affecting the gastrointestinal tract.