目的　提高临床医生对鼻腔鼻窦肌上皮癌的认识。方法　回顾性分析我科近期收治的两例鼻腔鼻窦肌上皮癌临床资料 ,并复习文献。结果　 1例为女性 ,4 7岁 ;1例为男性 ,5 4岁。临床表现为鼻塞、鼻腔暗红色肿物。鼻窦CT示鼻腔占位性病变。术前病理及免疫组化 (S - 10 0蛋白、平滑肌动蛋白、细胞角蛋白、波形蛋白等呈阳性染色 )确诊“肌上皮癌”。两例均行鼻侧切开鼻腔肿瘤切除术 ,例 1术后 5月出现颈淋巴结转移 ,术后 11月死于全身骨及肝转移。例 2术后 8月死于肺转移。结论　鼻腔鼻窦肌上皮癌极为罕见 ,迄今为止 ,仅 8例报道。综合文献 ,该瘤具有生长迅速、广泛侵犯周围组织、颈部淋巴结转移率高、血行转移率高、治疗后易复发、预后差等特点。诊断依赖病理及免疫组化。治疗以根治性手术为主 ,对于cN0的患者 ,应行分区性颈淋巴结清扫术 Objective To improve clinicians’ understanding of nasal sinus myoepithelial carcinoma. Methods The clinical data of two cases of nasal sinus myoepithelial carcinoma recently treated in our department were reviewed retrospectively. Results One case was female, 47 years old; one case male, 54 years old. Clinical manifestations of nasal congestion, nasal dark red mass. Sinus CT showed nasal cavity occupying lesions. Preoperative pathology and immunohistochemistry (S - 100 protein, smooth actin, cytokeratin, vimentin and other positive staining) confirmed "myoepithelial carcinoma. Nasal resection of the nasal cavity in both cases resection of the tumor, case 1 cervical lymph node metastasis occurred after 5 months, died of systemic bone and liver metastasis after 11 months. Case 2 died of lung metastasis in August after surgery. Conclusion Nasal sinus myoepithelial carcinoma is extremely rare, so far only 8 cases were reported. Comprehensive literature, the tumor has rapid growth, extensive invasion of surrounding tissue, cervical lymph node metastasis, high hematogenous metastasis rate, easy to relapse after treatment, poor prognosis and so on. Diagnosis depends on pathology and immunohistochemistry. Radical treatment is the main treatment for patients with cN0, zonal neck lymph node dissection should be performed