A 48-year-old woman was admitted to our hospital because of anemia since several years and abdominal distension since a month. Physical examination revealed anemia, jaundice, giant splenomegaly reaching 14 cm below the left costal margin, and slightly decreased vibration sense of lower extremities. Blood examination disclosed pancytopenia. Bone marrow aspiration showed erythroid hyperplasia with megaloblastic changes. The liver function tests were within normal limits except for a slight increase in indirect bilirubin and a marked increase in LDH. Decreased vitamin B12 absorption with correction by oral intrinsic factor and methylmalonic aciduria confirmed a diagnosis of pernicious anemia. She also had the positivity for the direct and indirect antiglobulin tests, which became negative 10 days after the start of the parenteral administration of vitamin B12. Following the therapy, anemia improved, but the recovery of leukopenia and thrombocytopenia was incomplete. The size of spleen has reduced to 7 cm below the costal margin by three months but did not change thereafter. One year after the diag nosis, splenectomy was performed. The portal pressure was normal. Histological examination of the spleen revealed chronic congestion, and that of the liver showed no abnormalities. The pancytopenia subsided soon after the operation. The significance of the splenomegaly and positive antiglobulin tests observed in this patient was discussed.