目的:该文旨在探讨乳腺恶性叶状肿瘤的临床表现、鉴别诊断及治疗现状。方法:对青岛大学附属烟台毓璜顶医院乳腺外科收治的1例乳腺恶性叶状肿瘤伴骨肉瘤分化的患者进行回顾性分析并复习相关文献。结果:乳腺恶性叶状肿瘤是一种含有腺上皮成分和间叶成分的双相性肿瘤,其发病率较低。针对乳腺恶性叶状肿瘤,尤其是伴有异源性分化者,应注意鉴别诊断,最终确诊依靠石蜡病理。手术为首选的治疗方法,且手术边缘的宽度与局部复发有关。Objective: The aim of this article is to investigate the clinical manifestations, differential diagnosis and treatment status of malignant phyllodes tumor of the breast. Methods: A case of malignant phyllodes tumor of the breast with osteosarcoma differentiation admitted to the Department of Breast Surgery of Affiliated Yantai Yuhuangding Hospital of Qingdao University was retrospectively analyzed and relevant studies were reviewed. Results: Malignant phyllodes tumors of the breast are biphasic neoplasms containing both epithelial and mesenchymal components, with a relatively low incidence. For malignant phyllodes tumors, particularly those exhibiting heterologous differentiation, careful differential diagnosis is essential, with definitive diagnosis relying on paraffin-embedded pathology. Surgical excision is the preferred treatment, and the width of surgical margins is closely associated with the risk of local recurrence.