This article reviews pediatric eosinophilic esophagitis (EoE), a clinicopathologic disorder with symptoms related to esophageal dysfunction and characterized by eosinophil-predominant inflammation present on esophageal biopsy. As part of this review, we outline the pathophysiology, clinical features, diagnostic criteria, approved treatment options, and emerging therapies of EoE. Although previously considered a rare condition, EoE has emerged primarily in Western countries, with an estimated incidence of 4 per 100,000 persons. Understanding disease manifestation, diagnosis, and treatment options are critical for primary care physicians and specialists alike as EoE presentation varies by patient age. Several treatment options are approved for first-line management and can be used interchangeably based on symptoms and patient preference. Additionally, the collaboration of gastroenterologists, allergist-immunologists, and nutritionists is recommended for a multidisciplinary approach to long-term EoE management. .