Objective To investigate the clinical characteristics of malignant triton tumor, so as to improve the diagnostic level. Methods The clinical data of 2 patients with malignant triton tumor was analyzed retrospectively with the literature review. Results The tumors occurred in the left tonsil and right parapharyngeal space respectively. One patient received from resection form the left lateral pharyngeal space via jaw and neck approach, combined with radiotherapy and chemotherapy postoperatively. The patient was followed up for more than 5 years after operation with a fine prognosis.This patient has slight limitation of mouth opening only and the daily life is independent. One threeyearold patient abandoned any treatment. Conclusion Malignant triton tumor is a rare subtype of malignant peripheral nerve sheath tumor with high invasiveness, and easy recurrence and metastasis. Pathological examination is the only means for diagnosis. Radical tumor resection combined with postoperative radiotherapy and chemotherapy may prolong MMT survival time.