The primary sarcoma of breast is a rare malignant tumor, which develops from the mesenchymal tissue of mammary gland. It represents less than 1% of all malignant diseases of breast. The incidence is about 17 new cases per 1 000 000 women. The etiology of that disease is unknown. The main method of treatment is the surgical excision with includes ";;;;safety-margin";;;; of healthy-looking tissues. We present a 57-year-old woman with probably familial, primary, malignat phyllodes sarcoma.