The case of a 39-year-old woman presented with symptoms of progressive cerebellar degeneration for few months preceding by the diagnosis and treatment of breast cancer. The causative association was revealed with the strong positivity of antineuronal antibody. Because of the multidisciplinary team approach with surgery and adjuvant endocrine treatment, the patient did improve symptomatically and she is alive without any evidence of disease after 22 months following the initial diagnosis of the neurological disorder. Interestingly, neurological symptoms regressed partially after surgery. Early recognition and appropriate combined modality treatment of this rare presentation of breast cancer are imperative as it may be crucial for the disease outcome.