To analyze the clinicopathological features of teratocarcinosarcoma of the nasal cavity, we presented a case of 54-year-old man and reviewed the literatures. A 54-year-old man presented to an outside institution with a 2-week history of nasal obstruction and epistaxis. A polypoid mass was found in his right nasal cavity. He subsequently underwent endoscopic sinus surgery. Histopathologic examination identified the malignant tumor was composed by two parts, one showing the histological features of carcinoma, the other with the features of sarcoma. There were nests of cells like neuroepithelium cells with scanty cytoplasm, inconspicuous nucleoli. Areas of nonkeratinizing squamous epithelium were found, there were also glandular structures lined by benign-appearing epithelium. Despite the epithelium component, the spindle cells showed the features of sarcoma with the apparent atypia and high mitosis index of 14/10 HPF. Benign cartilage and ciliated epithelium were also found in the tumor which revealed the features of teratoma of this malignant tumor. By immunohistochemistry, the epithelial components were positive with CK. NSE was positive in the neuroepithelium with focal positive pattern, so was CgA with a dotted positive pattern. The spindle cells were only positive with vimentin. A diagnosis of teratocarcinosarcoma was made. The patient accepted radio therapy after the surgery and was event-free in the next 30 months in follow-up. Teratocarcinosarcoma is a rare malignant tumor of nasal cavity which is needed aggressive therapy. The diagnosis depends on histopathology, the characteristic morphologic features and typical immunohistochemical phenotype can facilitate the diagnosis.