Eosinophilic esophagitis (EoE) is an immune-mediated disease that presents with dysphagia and esophageal bolus obstruction and is characterized by predominant intraepithelial eosinophilic infiltration at histology. The aim of our literature review was to delineate clinical and morphological EoE features in terms of differential diagnosis with other esophageal diseases, i.e., gastroesophageal reflux disease (GERD), inflammatory bowel diseases (IBDs), autoimmune diseases, and rare esophageal disorders. Clinical features of dysphagia and esophageal bolus obstruction and endoscopic criteria (according to EREFS) are typical of EoE. Nevertheless, eosinophilia of the esophageal mucosa is not a specific marker of mucosal injury and is not sufficient for EoE diagnosis. Therefore, eosinophilic esophagitis histological scoring system (EoEHSS) was developed. It involves assessment of intraepithelial eosinophilic infiltration, basal zone hyperplasia, eosinophilic abscesses, eosinophil surface layering, dilated intercellular spaces, surface epithelial alteration, dyskeratotic epithelial cells, and lamina propria fibrosis. Complex clinical, endoscopic, and histological evaluation allows accurate diagnosis of EoE and other causal factors of esophageal eosinophilia. Keywords: eosinophilic esophagitis, esophageal eosinophilia, gastroesophageal reflux disease, celiac disease, inflammatory bowel diseases, achalasia, atopy