There is an increased understanding and recognition of paraneoplastic syndromes in patients with a variety of malignancies. Paraneoplastic syndromes are associated with many tumor types and can potentially affect most organ systems. Breast cancer is one of the most common cancers worldwide, including in the USA, and can be associated with a variety of paraneoplastic syndromes affecting many systems, including the endocrine, neurologic, dermatologic, rheumatologic, hematologic and psychiatric systems. These syndromes can cause significant morbidity; therefore, effective diagnostic and treatment strategies need to be applied to improve quality of life, enhance delivery of cancer therapy and potentially prolong survival. This review focuses on the diagnosis and treatment of major paraneoplastic neurologic syndromes associated with breast cancer.