Background/Aim: Intramedullary spinal cord metastases (ISCM) are deemed extremely aggressive, as confirmed by the low life expectancy since the diagnosis. Up to 26.5% of total ISCM stem from breast cancer (BC), representing the second most frequent primary site after the lung. The increasing incidence of BC and the widespread use of MRI for the diagnosis could therefore lead to an earlier diagnosis and, therefore, to a progressively longer survival in patients affected by ISCM from BC. This systematic review is intended to provide an orientation through a management algorithm for the most appropriate therapeutic approach in these patients. Materials and Methods: The research strategy initially relied on title and abstract analysis. The article's full text was retrieved for further investigation if the title and abstract met the inclusion criteria. The extracted data included the following: authors, publication time, study design, patient characteristics, ISCM location, treatment modalities, time interval from initial cancer diagnosis to ISCM diagnosis, clinical outcomes, and survival time. Results: This systematic search regarding ISCM from BC yielded 574 articles. After screening, a total of 44 studies were included in this systematic review. A total of 123 patients were evaluated. The mean patient age was 53.2 years with a standard deviation of 10.4 years. Female patients were 122. There was only one male patient. Conclusion: ISCM from BC have a better prognosis than lung metastases and, thanks to recent advances in diagnostic imaging and intraoperative planning and neuromonitoring, an early diagnosis and a prompt multidisciplinary treatment may be accomplished. Prospective studies to generate evidence-based data regarding the most appropriate treatment for ISCM are mandatory.Remote metastases to the central nervous system are common, and spinal cord involvement represents a small percentage of all the involved sites. Autopsy series performed on cancer patients determined an average frequency of intramedullary spinal cord metastases (ISCM) below 2% (1-3). ISCM are characterized by a rapid neurological impairment and progression, as they exert highly malignant tumor evolution. Notably, ISCM are deemed extremely aggressive, as confirmed by the low life expectancy since the diagnosis, which ranges from 3 to 12 months (2, 4, 5). Yet, on average, approximately 8% of the patients are asymptomatic at the time of diagnosis (4, 6). Once early signs appear, they typically advance quickly and progress into a sensory and/or motor deficit within 4 weeks (6-8).Hence, early diagnosis and prompt treatment are pivotal to improve the prognosis and the neurological condition of these patients (6). Therefore, when a spinal involvement is suspected, patients with a previous oncological history, should undergo a high-resolution spine MRI with Gadolinium enhancement (8).