Background: Malignant fibrous histiocytoma (MFH) is a malignant type of sarcoma with uncertain origin and it may arise from bone and soft tissue. In 3% cases it involves head and neck and it is rare in larynx. Case report: We present a rare case of a 55-year-old male diagnosed as pleomorphic sarcoma (PS) of larynx. Conclusion: Plemorphic sarcoma, commonest subtype of MFH, should be kept in mind as a differential diagnosis in aggressive tumors of larynx. Clinical significance: Pleomorphic sarcoma of larynx may be diagnosed as rare tumor of larynx and may need aggressive treatment.