Primary Sjogren's Syndrome(pSS) is a chronic autoimmune disease and focal lymphocytic infiltration of the exocrine glands is the key pathological feature (1). Polycythemia vera(PV) is a chronic myeloproliferative disorder characterized by JAK-2 mutations and increased red blood cell mass(2,3). Autoimmune diseases and hematological malignancies can often be observed together. Patients with SS are at a higher risk of developing lymphoproliferative diseases mainly low-grade B-cell lymphomas and predominantly marginal zone lymphoma. Moreover, PV can rarely be presented with paraneoplastic events including small vessel vasculitis (2). However, PV and pSS co-existence had never been reported. We here want to present a case of concurrently diagnosed PV and pSS.