The eosinophilic gastrointestinal diseases (EGIDs) represent disorders of the gastrointestinal (GI) tract that result from the local infiltration and aberrant activity of eosinophils and other immune cells. Eosinophilic esophagitis (EoE) is the most well-characterized EGID and is defined by the presence of intraepithelial eosinophils in the esophagus (≥15 eosinophils per high-powered field) and clinical symptoms associated with esophageal dysfunction. The other EGIDs are rare and lack strong data regarding pathogenesis and management. The incidence and prevalence of EoE are increasing, and EoE is now a major cause of upper GI morbidity. Management is multidisciplinary, with collaboration between gastroenterologists, allergists, pathologists, and dieticians, and is aimed at amelioration of symptoms and prevention of long-term complications such as esophageal stricture. Treatment options for EoE include proton pump inhibitors, swallowed topical corticosteroids, and elimination diets. Esophageal dilation is used when esophageal strictures or fibrostenotic changes are present. Additional therapies targeting eosinophils and other mediators of Th2 inflammation are under development and are promising. Treatment options for other EGIDs typically involve corticosteroids or dietary elimination.