Neuroendocrine breast cancer was defined in 2003 by WHO as a separate and unique breast cancer subtype. Primary small cell carcinoma of the breast, an exceptionally uncommon and aggressive tumor, is frequently characterised by early progression and worse outcome. Moreover, it is essential to distinguish between small cell carcinoma arising primarily in the breast and the metastatic disease to the breast. We had a patient of primary small cell carcinoma of breast. As her initial metastatic workup was negative for disease elsewhere, so she was started on neoadjuvant chemotherapy to which she responded well. Her modified radical mastectomy (MRM) was done followed by completion of chemotherapy up to 6 cycles and local radiotherapy of chest wall. However, the disease behaved aggressively afterwards as she developed recurrence twice at 9 and 19 months interval, respectively, for which she was considered for second and third line chemotherapy. An accurate management of the primary small cell carcinoma of the breast still lacks a consensus. Relevant studies were also reviewed to enhance knowledge and expertise in diagnosis, clinicopathologic features, management, and outcome of this tumor.