Angioimmunoblastic T cell lymphoma (AITL) is a rare subtype of T cell non-Hodgkin lymphoma. The standard therapeutic strategy for AITL has not yet been established, and its prognosis remains poor. This report concerns the effect of cyclosporin A (CsA) on chemotherapy-refractory AITL. A 68-year-old female with AITL with systemic symptoms, such as high fever, skin rash andgeneralized lymphadenopathy, was initially treated with conventional cytotoxic chemotherapies using alkylators, anthracyclines and corticosteroids, which failed to induce remission. However, CsA (4 mg/kg/day) plus dexamethasone treatment resulted in a dramatic regression of the tumors and amelioration of systemic symptoms and induced complete remission (CR) within 2 weeks. Currently, the patient’s CR has continued for more than 18 months with CsA maintenance therapy. Our experience and previously reported findings suggest that CsA may constitute an alternative treatment option for AITL, even though the use of conventional cytotoxic chemotherapy continues to be the first-line therapy on an empirical basis.