Sjögren’s syndrome (SS) is a systemic autoimmune disease that presents with sicca symptoms of the main mucosal surfaces. The spectrum of disease extends from sicca syndrome to systemic involvement and may be complicated by the development of lymphoma. Many types of malignant and pseudo malignant diseases have been reported, but the most important is non Hodgkin lymphoma. We here present a 45 year-old woman with SS that referred with pancytopenia. By molecular and histochemical techniques a diagnosis of acute myeloblastic leukemia of M3 type (AML-M3) was made for this patient.