A 31-year-old female with Primary Sjögren Syndrome (pSS) presented with bilateral puffiness around the eye for 3 years. The lacrimal glands were hypertrophic and edematous bilaterally. Schirmer 1 score was 2 and 1 mm and tear-film break-up time was 3 and 4 seconds, in the right and the left eyes, respectively. An incisional biopsy from the left lacrimal gland revealed diffuse and intense CD20, CD5, and bcl-2 positivity with negative cyclin D1 and CD23 which supported lymphoma. Upon haematology consultation extranodal marginal zone lymphoma diagnosis was made. CHOP (cyclophosphamide, doxorubicin, vincristine, and methyl prednisolone) treatment was initiated. In conclusion, pSS is a well known autoimmune disease in which increased rate of lymphoma is present. Early detection with histopathologic confirmation and multidisciplinary approach with ophthalmology, rheumatology, and haematology are mandatory in these patients.