Low grade fibromatosis-like spindle cell metaplastic carcinoma (FLSpCC) of the breast is a very rare variant of breast neoplasia presenting characteristic clinical and biological features: atypical clinical and radiologic presentation amd high risk of local recurrence but low risk of lymph node and distant metastases. Differentiation from other malignant tumors can be obtained only by histologic and immunohistochemical evaluation. These particulars may render the diagnosis and surgical management of such tumors challenging. Given the small amount of data published, every clinical case reported may be important to improve our knowledge on the argument. Therefore we describe in detail the clinical, diagnostic, histopathologic, and therapeutic information observed in our patient and compare them with those acquired by a review of the literature. Of particular interest is the comprehension of the role of the surgical resection margins for the reduction or elimination of local recurrences, which represent the most fearful aspect in the management of such tumors. Another interesting aspect is the assessment of the appropriate follow-up strategy for the early detection of local recurrences or distant metastases. The latter is rare but it must always be considered in patients with FLSpCC.