Marginal Zone Lymphoma (MZL) accounts for between 5% and 17% of all non-Hodgkin lymphomas (NHL). We reported a rare case of marginal zone lymphoma presenting with bilateral ear erythema and swelling. The case was a 79-year-old man who suffered from progressive bilateral ear erythema and swelling since 2 years ago. Physical examination was remarkable for erythmatous, edematous and non-tender auricles bilaterally which affected both lobules. He scheduled for a punch biopsy and pathology report revealed some atypical cell with large vesicular nuclei, prominent nucleoli, with clear cytoplasm (monocytoid B-cells) in mid-dermis with extension to deep dermis favoring low grade B-cell lymphoma, marginal zone subtype. A computed tomography of the chest revealed lymphadenopathy in both hilar, subcarina, aortopulmonary and retrocaval space greater than 10 millimeter. In conclusion, Involvement of the ears is an unusual finding in malignancy and therefore tissue biopsy must perform, even it is bilaterally.