A 53-year-old-male patient was admitted to the Hiroshima University Hospital in August 2001, with a history of progressive cerebellar ataxia, notable by standing and gait disturbances. Brain computed tomography (CT) and magnetic resonance imaging (MRI) showed no signs of ischemic damage. Two weeks later, left axillary lymphadenopathy developed. The pathological finding was Hodgkin's disease (HD; a classical malignant lymphoma in an early stage). Serum antibodies against Purkinje's cells (anti-Tr antibody) were detected by immunohistochemical study. Thus, we diagnosed this as paraneoplastic cerebellar degeneration (PCD) associated HD. We immediately carried out chemotherapy (ABVD therapy), radiotherapy with 20-30 Gy, steroid pulse and intravenous immunoglobulin (IVIg) without neurologic improvement. The patient was subsequently treated by double filtration plasmapheresis (DFPP), leading to a marked improvement of standing and gait disturbances and to a reduction of autoantibodies, which became undetectable. We conclude that DFPP might be useful in treating patients with cerebellar ataxia, and should be considered as a therapeutic choice for it.