Eosinophilic gastroenteritis is an infrequently diagnosed condition that is characterized by prominent eosinophilic infiltration of the stomach or small intestine, generally localized to one level of the intestinal wall; the variable organ locus and wall depth produce heterogeneous clinical presentations. A strong association with atopy is present in most cases, supported by circumstantial evidence and the demonstration of Th-2 proinflammatory cytokine profiles in animal studies. A high degree of suspicion is required to establish the diagnosis, which must be based on intense gastrointestinal eosinophilia. Management is directed toward removal of offending allergens and use of anti-inflammatory agents. Novel and emerging treatments on the horizon are biologic therapies and selective anti-eosinophil agents.