Primary marginal zone lymphoma of the esophagus is a rare disease, the causes of which may be related to immune abnormalities. The article describes the case of a 68-year-old man who had a ten-day history of cough, excessive sputum production, and wheezing, having sought medical attention two years previously for fever and cough. Computed tomography of the chest revealed thickening of the esophageal wall in several segments and airway compression. Endoscopic examination showed an extensive protruding lesion along the entire esophagus with a smooth surface without wrinkles. Histopathological and immunohistochemical examinations confirmed the diagnosis of marginal zone lymphoma. This case is exceptional because it presented with respiratory symptoms without typical swallowing difficulties, which increases the risk of misdiagnosis. The patient was treated with two courses of the G-CVP regimen with suboptimal efficacy, then switched to the triple combination of Obinutuzumab, Orelabrutinib and Lenalidomide, which completed six cycles.