Eosinophilic esophagitis (EoE) is a chronic immune-mediated condition where infiltration of eosinophils into the esophageal mucosa leads to symptoms of esophageal dysfunction. It has rapidly emerged as an important cause of upper GI morbidity in patients of all ages and is encountered in a substantial proportion of patients undergoing diagnostic upper endoscopy. This review discusses the clinical, endoscopic, and histologic features of EoE and presents the most recent guidelines for diagnosis of EoE. It describes selected diagnostic dilemmas including distinguishing EoE from gastroesophageal reflux disease and addressing the newly recognized clinical entity of proton pump inhibitor responsive esophageal eosinophilia. It also highlights evidence to support both pharmacologic and non-pharmacologic treatments, including topical corticosteroids, dietary elimination therapy, and endoscopic dilation.