Background/Objectives: Lymphocytic esophagitis is a rare inflammatory disease of the esophagus, first described in 2006. Since that time, interest in this disease entity has steadily increased, yet specific data on the etiology of the disease are still lacking. Lymphocytic esophagitis co-occurs with esophageal motility disorders, Crohn’s disease, hypersensitivity (allergy, asthma), the use of certain medications, or immune disorders. Risk factors include smoking and alcohol abuse. The disease is more prevalent in women, developing predominantly within the sixth decade of life. The most important symptom is dysphagia, with odynophagia, epigastric, and chest pain or heartburn being less common. No particular endoscopic presentation is pathognomonic for lymphocytic esophagitis, the findings not infrequently resembling those of eosinophilic esophagitis. Conclusions: There is great need for an international consensus defining strict histopathological criteria for the diagnosis of lymphocytic esophagitis as well as relevant diagnostic and therapeutic management. This review paper presents the current state of knowledge on lymphocytic esophagitis.