Marginal zone lymphomas are a group of slow-growing non-Hodgkin B-cell lymphomas. This type of lymphoid tissue tumors occur extremely rare in larynx. Since the first description by Diebold et al in 1990, less than 50 cases marginal zone lymphomas of the larynx have been reported [1, 2]. We present a case report of a 66-year-old woman, admitted in April 2012 to an ENT Ward in Polanica Zdroj hospital in order to perform a biopsy of lesion present in larynx since at least 2 years. Patient presented with hoarseness, mounting dyspnoea and history of recurrent inflammation of larynx and trachea. Histopathologic examination revealed B-Cell Marginal Zone Lymphoma. The patient was sent to undergo further hematological treatment (R-CHOP regimen chemotherapy), which was followed by a complete remission. It is important to emphasize that it was the 3rd biopsy of the lesion taken in 2 years time (since 2010) preceded by two such procedures in another clinical ENT Ward in Poland. The microscopic examination results of the previous biopsies were described as indicative more of an inflammatory lesion although both of them were said to be hard to evaluate. This shows the diagnostic difficulties that biopsy itself or its microscopic interpretation might bring.