Eosinophilic gastroenteritis is a clinicopathological disease affecting both children and adults that is characterized by patchy or diffuse eosinophilic infiltration of the gastrointestinal tract with variable resultant clinical gastrointestinal manifestations. The eosinophil, eotaxin, and Th-2 cytokines are important in pathogenesis of this disease entity. It may be confused with parasitic and bacterial infections (including Helicobacter pylori), inflammatory bowel disease, hypereosinophilic syndrome, myeloproliferative disorders, periarteritis, allergic vasculitis, scleroderma, drug injury, and drug hypersensivity. Obtaining the correct diagnosis is important, and a pathologist usually makes this distinction. Effective treatments include systemic/topical corticosteroids, specific food elimination or an elemental diet, certain drugs, and even surgery. A variety of new therapeutic approaches are now under trial.