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Sinonasal teratocarcinosarcoma (SNTCS) is a highly invasive malignant tumor most frequently found in the nasal cavity and paranasal sinuses. As a result, it can be confused with other sinonasal tumors. In addition, SNTCS progresses rapidly and often infiltrates other tissues or organs in the early phase, resulting in poor patient prognosis. The objective of this article was to report the case of a patient with SNTCS and discuss the management strategy. Furthermore, we conducted a literature review for SNTCS and summarized the findings fro