Primary nasopharyngeal lymphoma is a very rare tumor with a variety of histopathologic subtypes, with only limited studies reported in literature. Sharing an unusual case report of primary nasopharynx extranodal marginal zone B-cell lymphoma, low-grade, as well as its diagnostic and therapeutic outcomes. A case report. A 46-year-old male was admitted to our clinic reporting nasal congestion and ear whistling. Negative history of any other chronic diseases or cancer. Physical examination revealed no pathological findings. The following were the laboratory findings: SE:4, WBC:6.3 103/mm3, Er:5.7 106/mm3, Hgb:14.6g/dl, Hct:48.6%, Plt:180×103/mm3, Mcv:85µm3, MCH:25.3pg, MCHC:29.3g/dL, Ret:3promil, and LDH:375U/l. The abdomen, axilla, and inguinal ultrasound scans were also normal, but the ultrasound scan of the neck revealed two hyperechoic nodules 23×24mm in the thyroid gland. On a CT scan of the paranasal spaces, maxillary sinusitis, nasal septum deviation, and polyposis in the nasopharynx were discovered and then a biopsy was performed on that site. The histopathology and immunohistochemistry diagnosis revealed a low-grade extranodal marginal zone B-cell lymphoma, positive on CD3, CD20, Bcl-2, CD10, Pax-5, CD23, MUM-1, CD138, and Ki-67:5 %. We decided to begin with chemotherapy rituximab, cyclophosphamide, hydroxydaunorubicin hydrochloride, vincristine, and prednisone (R-CHOP) protocol every 21 days. After four treatments, the CT scan revealed a free nasopharynx and a thyroid nodule that was partially shrunk, size 17×18mm, and then we decided to continue with four more R-CHOP treatments. A repeated CT scan revealed free paranasal spaces and in the ultrasound scan, there were still thyroid nodules 22×24mm. PET-CT examination revealed no pathological accumulations after a total of eight R-CHOP protocol treatments. For further examination, thyroid scintigraphy and cytological analysis-FNA were recommended. Thyroid hormones, anti-TPO, anti-TG, calcitonin, and CEA levels were in the norm. The patient is currently waiting for a thyroid nodule biopsy. 14 months after completing the R-CHOP treatment protocol, the patient is still in remission, with no signs of disease and a normal CT scan. This type of tumor's prognosis is difficult to predict, and there are far too few examples to draw firm conclusions.