ObjectiveThe incidence of sinonasal malignancies (SNM) is relatively low, the early symptoms and signs are not specific. The most OF SNM are characterized by strong invasiveness, easy lymph node metastasis and distant metastasis, and poor prognosis. Therefore, it is difficult to make clinical diagnosis. There are various pathological types of SNM, which are more than 60% for squamous cell carcinoma, followed by adenoma. At present, most reports on SNM are studies of single pathological type， and comprehensive studies on multipathology of SNM are lacking. In this paper, we reviewed the clinical features, histopathology, molecular genetics and comprehensive treatment of Biphenotypic sinonasal sarcoma (BSNS), Sinonasal teratocarcino sarcoma (SNTCS), NUT midline carcinoma (NMC), small cell neuroendocrine carcinomas(SNEC) and other rare SNMS.