A 64-year-old male was diagnosed as having autoimmune hemolytic anemia (AIHA) in December 2001. The diagnosis of AIHA was established based on the presence of anemia, hyperbilirubinemia, reticulocytosis, positive Coombs test and serum LDH elevation. Although he received steroid pulse therapy, disturbance of consciousness appeared and the anemia and icterus did not improve. Plasma exchange was carried out to eliminate antibodies as an emergency measure. The patient's symptoms were ameliorated following four courses of plasma exchange. This case represents a case of aggressive AIHA with disturbance of consciousness and refractory to conventional corticosteroid therapy. Plasma exchange rapidly led to complete remission.