We experienced a remarkable effect of recombinant interferon alpha 2a (alpha-IFN) in a case of angioimmunoblastic lymphadenopathy with dysproteinemia (AILD) which was refractory to steroids and combination chemotherapies. A 62 year-old woman was admitted because of high grade fever and extreme swelling of cervical lymph nodes. Poly-clonal hypergammaglobulinemia and plasma cell-like atypical lymphocytosis in the peripheral blood were demonstrated. Cervical lymph node biopsy disclosed histology of AILD. She initially responded well to prednisolone. Three months later, AILD relapsed in spite of prednisolone treatment. She received combination chemotherapies and responded well again. Seven months later, she became refractory to these combination chemotherapies. Consequently, we tried alpha-IFN (3 million units/day given intramuscularly). She became afebrile on the next day, and lymph nodes swelling gradually disappeared. She has been free from the disease for more than three months.