MFH is a pleomorphic sarcoma first described by O'Brien and Stout in 1964. This tumor has been usually found in the muscles of the limbs and retroperitoneum, more uncommonly in the head and neck, and extremely rarely in the nasopharynx. The great majority of cases of MFH in the head and neck present with a painless mass, have a peak incidence in the seventh decade and are distinctly uncommon before the fourth decade of life. Occasionally, synchronous tumors have been noted. The authors recently experienced a case of giant nasopharyngeal MFH combined with malignant transformed inverted papilloma in the nasal cavity in sixty-five years old man causing acute respiratory distress which required a preoperative emergency tracheostomy.