Paraneoplastic cerebellar degeneration (PCD) is a debilitating neuro-degenerative disease associated with antibodies directed against the purkinje cells of the cerebellum. Treatment using chemotherapy or other treatment of the primary tumor to various immunologically directed therapies has been attempted but outcomes have been poor. We discuss a patient with ovarian carcinoma and PCD seen in our institution who showed a marked beneficial response to intravenous immunoglobulin (IVIG) and methylprednisolone. A Medline search from 1966-2002 produced fifteen cases of PCD confirmed by antibody testing that were treated with IVIG, either alone, or with a combination of other therapies. The clinical characteristics and treatment responses of these patients are analyzed in this review. Most patients that were treated with IVIG and had what was defined as a good response were treated within one month of symptoms. Patients treated between one month and three months often had stable disease and patients treated after three months of symptoms usually had a poor outcome. Early treatment with sufficiently high doses of IVIG seems to provide a better chance of treatment success. The additional benefit of early high dose intravenous methylprednisolone is unclear. Due to the devastating nature of the disease, a trial of IVIG and steroids is warranted as early as possible in a dose of 2g/kg to any patient with a clinical picture of PCD and positive antibodies.