Food Protein-Induced Enterocolitis Syndrome (FPIES) is an uncommon and potentially severe form of non-IgE-mediated food allergy. Two clinically distinct forms have been reported. In the chronic or early form symptoms typically begin in the first month of life, while the antigen is being ingested on a regular basis, with chronic diarrhoea and intermittent vomiting, in association with failure to thrive. In the acute or late form symptoms may occur at an older age with profuse, repetitive vomiting, often with diarrhoea, that leads to acute dehydration, lethargy and, in the most severe cases, shock and hypotension. The acute manifestations usually start two or more hours after the ingestion of the offending food. FPIES is typically caused by milk or soy in formula-fed infants, but may also be triggered by ingestion of solid foods such as rice, oat, barley, fish, and others. Frequently children with FPIES reacted to more than one food. The majority of patients have negative skin prick tests and undetectable food-specific IgE, thus the diagnosis of FPIES is usually made on a clinical basis. The pathophysiology is still unclear; however FPIES seems to be a cell-mediated immunologic disorder in which TNF-alfa may play a key role. For the acute phase, treatment with intravenous hydration is often needed. Avoidance of the trigger food protein/s is currently the only effective treatment option, however most children will outgrow their FPIES by about three to five years of age. Early recognition of FPIES remains a critical issue to prevent misdiagnosis and mismanagement of symptoms that may mimic other causes.