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Breast chondrosarcoma is a rare sarcoma that mainly occurs in females >50 years old. To the best of our knowledge, only 16 cases were reported in the literature prior to 2013 and all patients were surgically treated by mastectomy, with or without lymphadenectomy, which was occasionally preceded by neoadjuvant chemotherapy. However, the literature does not report the benefit of mastectomy compared with a more conservative surgery. The present study reports a novel case of extraskeletal chondrosarcoma of the breast. A 63-year-old female patient presented with a neoplasm localized in the upper-outer quadrant of the right breast. The palpable lesion with sharp margins was a firm parenchymatous mass, which was confirmed by ultrasonography and mammography. The patient underwent conservative quadrantectomy instead of mastectomy, followed by post-surgical chemotherapy. A positron emission tomography scan performed five months subsequent to the surgery revealed no remnants of the disease. The patient underwent a strict clinical and instrumental follow-up, and two and half years after surgery, there are no signs of recurrent disease. In conclusion, the present case is currently one of the two cases in which a more conservative quadrantectomy was performed, instead of mastectomy. This surgical approach did not lead to metastasis and resulted in a good follow-up for the patient. [ABSTRACT FROM AUTHOR]