A 59 year old female was diagnosed as having Sjögren's syndrome in 1963. A short time later, she developed a refractory sideroblastic anemia. In 1972, she was found to have a preleukemic state with a persistent "shift to the left" of the granulocytic series, terminating early in 1974 as acute myeloblastic leukemia. Although several cases of malignant lymphomata have been described in association with Sjörgen's syndrome, to our knowledge no other example of Sjörgen's syndrome has been described in association with a myeloproliferative disorder. Defective immune surveillance produced by the Sjörgen's syndrome may have permitted the development of the myeloproliferative syndrome. Alternatively, both disorders may have developed from a hemopoietic stem cell defect.