Sinonasal teratocarcinosarcoma (SNTCS) is a rare malignancy characterized by a highly aggressive nature. It mainly arises in the ethmoidal or maxillary sinus. SNTCS has a poor prognosis, with a mean survival rate of 55% at 2 years. Herein, we presented a case of advanced SNTCS successfully treated with surgery followed by chemoradiotherapy plus targeted therapy and reviewed the published literature on this rare entity.