目的:探讨下咽癌肉瘤的临床病理学特征、诊断和鉴别诊断。方法:报道1例经病理证实的下咽癌肉瘤患者的临床资料,结合文献复习进行分析。结果:患者通过手术切除病变,病理检查证实为鳞状细胞癌横纹肌肉瘤,术后行放化疗,恢复良好,随访18个月无复发。结论:下咽癌肉瘤是一种极为罕见的恶性肿瘤,具有特征性的组织病理学、免疫组织化学及临床特点,需与肉瘤样癌、喉癌的放疗反应等相鉴别。下咽癌肉瘤的治疗应首选手术切除。对局部晚期、术后残留、肉眼切除范围欠安全的头颈部癌肉瘤患者应行放疗并及时随访。 Objective: To investigate the clinicopathological features, diagnosis and differential diagnosis of hypopharyngeal carcinosarcoma. Methods: One case of pathologically confirmed hypopharyngeal carcinosarcoma was reviewed and analyzed in combination with literature review. Results: The patients were surgically removed and pathologically confirmed as squamous cell carcinoma of rhabdomyosarcoma. Chemotherapy was performed after operation. The patients recovered well and had no recurrence 18 months after follow-up. Conclusion: Hypopharyngeal carcinosarcoma is an extremely rare malignant tumor with characteristic histopathology, immunohistochemistry and clinical features. It needs to be differentiated from sarcomatoid carcinoma and laryngeal carcinoma in response to radiotherapy. Hypopharyngeal sarcoma should be the preferred surgical treatment. For locally advanced, postoperative residual macroscopic excision of the scope of patients with head and neck cancer should be treated with radiotherapy and timely follow-up.