A 71-year-old Japanese man was referred to our hospital with difficulty swallowing, monoclonal gammopathy, an increased white blood cell count (9,650/mm 3 ), atypical lymphocytes (40%), anemia (hemoglobin, 9.5 g/dL), thrombocy-topenia (platelet count, 12,800/mm 3 ), and an increased soluble interleukin-2 receptor level (1,378 U/mL). Computed tomography showed bulky, diffuse esophageal wall thickening (Picture 1, arrows), in addition to orbital tumors and intrapleural and intra-abdominal lymphadenopathies. The tracer uptake in the tumors was observed on positron emission tomography (Picture 2, arrows). Esophagogastroduodenoscopy revealed extensive mucosal thickening, resulting in the narrowing of the esophageal lumen (Picture 3). A conventional endoscopic biopsy of the esophageal lesion using biopsy forceps led to the diagnosis of stage IV extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT) lymphoma showing small-to mediumsized atypical lymphoid cells, which were positive for CD 20, BCL2, and immune receptor translocation-associated protein 1 (IRTA1) and negative for CD3 and CD10 (1). The patient was treated with rituximab plus chemotherapy, which resulted in partial remission. In the present patient, it was noteworthy that the size of the lymphoma lesion was largest