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Introduction and importance DFSP is a soft tissue sarcoma that originates from dermal fibroblasts, that usually occurs in trunk or extremities and can invade the subcutaneous tissue. It rarely occurs in the breast with an incidence of 0.8–4.5% % per million population. It usually presents as a firm, well-defined, mobile, nontender mass. The diagnosis is confirmed by histopathology and immunohistochemistry. The recommended treatment is wide local excision. Adjuvant radiotherapy or imatinib should be considered in special cases. The recurrence-free survival and overall survival are good. Case presentation In this study, we present a 48-year-old woman with a growing tumor in her left breast. Clinical discussion The patient underwent breast-conserving surgery and reconstruction. Conclusion The diagnosis of DFSP was confirmed after a precise histological assessment. Highlights