Malignant triton tumors (MTTs) are a rare subtype of malignant peripheral nerve sheath tumor (MPNST) showing rhabdomyoblastic differentiation, which have no treatment consensus and a poor prognosis. This case report presented the case of a 42-year-old male patient with a large MTT located in the anterior mediastinum. The patient underwent palliative chemoradiotherapy and interstitial chemotherapy and received traditional Chinese medicine. Localization of an MTT in the anterior mediastinum is extremely rare. To the best of our knowledge, this is only the fourth study of an MTT localized in the anterior mediastinum that has been documented in the English literature.