Neuroendocrine breast cancers are rare tumors that were recognized as a distinct entity by WHO classification in 2003. It is much rarer in male breast cancer. Diagnosis is based on immunochemical analysis in which the expression of at least one neuroendocrine marker is required, associated with the exclusion of another primary site of the tumor. These tumors have a worse long-term outcome compared to other breast cancers. Small cell carcinoma of the breast is a high-grade subtype, presents with more advanced disease, and has a poorer prognosis compared with other neuroendocrine breast subtypes. A proper therapeutic strategy is still not well established. In the herein reported case, a 62-year-old male patient was diagnosed with small cell neuroendocrine carcinoma of the breast, metastatic to the liver, lung, bone and lymph node, and was treated with a first-line Platinum-Etoposide chemotherapy combination with a good clinical and radiological response. Only four previous cases of male small cell breast carcinoma were reported. Keywords: Neuroendocrine Breast Carcinoma, Small Cell Carcinoma, Diagnosis, Prognosis, Treatment.