目的　探讨鼻腔原发性透明细胞癌的临床病理学特征及诊断、鉴别诊断要点。方法　对 1例鼻腔透明细胞癌进行临床病理分析及免疫组织化学研究。结果　鼻腔透明细胞癌临床表现为进行性加重的鼻塞并出现间断排血性鼻分泌物。镜下瘤组织主要由多边形上皮样瘤细胞组成 ,排列成巢状 ,由富含薄壁毛细血管的纤维组织分隔。瘤细胞大小不一 ,核小 ,居中或偏向一侧 ,核分裂罕见 ,胞质丰富透亮 ,呈空泡状。免疫组化 :肿瘤细胞显示ker atin、S 10 0阳性 ,HMB 45、vimentin、actin、CGA、Syn、和α SMA阴性 ;纤维组织vimentin阳性。结论　鼻腔原发透明细胞癌是罕见的肿瘤 ,其诊断和鉴别诊断主要依靠病理组织学和免疫组织化学 Objective To investigate the clinicopathological characteristics of primary clear cell carcinoma of the nasal cavity and its diagnostic and differential diagnosis. Methods One case of clear cell carcinoma of the nasal cavity was studied by clinicopathological and immunohistochemical methods. Results The clinical manifestations of clear cell carcinoma of the nasal cavity were aggravated nasal congestion and intermittent discharge of nasal secretions. Microscope tumor tissue mainly by polygonal epithelioid tumor cells arranged in nests, separated by fibrous tissue rich in thin-walled capillaries. Tumor cells of different sizes, nuclear small, central or biased side, rare nuclear fission, cytoplasm rich translucent, vacuolar. Immunohistochemistry: tumor cells showed ker atin, S 10 0 positive, HMB 45, vimentin, actin, CGA, Syn, and α SMA negative; fibrous tissue vimentin positive. Conclusions Nasal primary clear cell carcinoma is a rare tumor whose diagnosis and differential diagnosis mainly rely on histopathology and immunohistochemistry