Food protein-induced enterocolitis syndrome (FPIES) is a non-IgE-mediated food hypersensitivity with usual onset in infancy. The most common FPIES triggers are cow milk, soy and rice; in addition, oats, vegetables, egg, poultry and seafood have been reported. In the acute form, when food is ingested on an intermittent basis or following a period of avoidance, FPIES presents with profuse vomiting within 1-3 h after ingestion, occasionally accompanied by diarrhea and dehydration. In the chronic form, when food is ingested on a regular basis, FPIES presents with intermittent vomiting, diarrhea, weight loss and failure to thrive. FPIES is diagnosed based on history and typical symptoms, which improve with food avoidance, and exclusion of other etiologies. Oral food challenge remains the gold standard for FPIES diagnosis. Most CM or soy FPIESs resolve within the first 3-5 years; solid food FPIES or FPIES associated with positive food-specific IgE may have a more protracted course. The prevalence of FPIES is unknown.