Autoimmune hemolytic anemia (AIHA) in Sjögren's syndrome (SS) is a rare occurrence. A patient presented with severe AIHA that seemed idiopathic, responded to treatment with prednisone and azathioprine but she developed symptoms and signs of the sicca syndrome within 8 months. This is a first report of AIHA preceding SS in an adult and the association is discussed. We believe that primary SS should be included in the differential diagnosis of acquired AIHA.