A case of hairy cell leukemia (HCL) presenting as autoimmune hemolytic anemia (AHA) is described. A 40-year-old woman presented with severe hemolytic anemia. The morphological and immunological studies of bone marrow and spleen revealed a hairy cell leukemia. Although autoimmune diseases are a well known complication of HCL this is the first description of AHA as a complication of HCL.