Esophageal lichen planus is an underrecognized condition, with fewer than 50 cases reported to date. Unlike cutaneous lichen planus, esophageal lichen planus occurs almost exclusively in middle-aged or older women who also have oral involvement. It commonly involves the proximal esophagus and manifests as progressive dysphagia and odynophagia. Endoscopic findings can include lacy white papules, pinpoint erosions, desquamation, pseudomembranes, and stenosis. Histologic features of esophageal lichen planus have only rarely been illustrated. They differ from those of cutaneous disease in several respects, including the presence of parakeratosis, epithelial atrophy, and lack of hypergranulosis. Correct diagnosis of esophageal lichen planus is difficult but bears important therapeutic implications. It is typically a chronic and relapsing condition that can require systemic or local immunosuppressive therapy and repeated endoscopic dilatations for esophageal strictures. Esophageal lichen planus may have malignant potential, as evidenced by 3 patients who developed squamous carcinoma of the esophagus after longstanding disease.