Sjogren Syndrome (SS) is an autoimmune disease characterized by a lymphocytic infiltration of exocrine glands. This abnormality leads to the destruction of these glands with the clinical expression of the sicca syndrome (dry eyes and dry mouth). Usually Sjogren Syndrome follows a non-aggressive course, but patients suffering from this disease have a very high risk of developing malignant lymphoma (MALT). The evolution from benign lymphocytic infiltration characteristic of Sjogren Syndrome to malignant lymphoma (usually MALT subtype) is a multistep process [1-3]. We present the case of 35 years old woman admitted to “Sf. Maria” Clinical Hospital—Bucharest, Romania, in the Rheumatology Department in May 2011, representative for the diagnosis difficulties as well as for the unfortunate evolution. She was diagnosed with primary Sjogren Syndrome in 2004. The first symptoms of the disease, dry eyes and dry mouth, appeared in 2000 but they were undermined by some hematological disorders, as the patient had a low white blood cell count at the biological evaluation. Four years later, when the diagnosis of Sjogren Syndrome was set, she already had some negative prognosis factors. In the end, the disease had an unfavorable outcome, as the patient developed non-Hodgkin malignant lymphoma for an unfortunate evolution.