Sarcomas of the larynx are exceedingly uncommon, of which primary laryngeal osteosarcomas are the rarest. To date, 25 cases of primary laryngeal osteosarcomas have been reported in the literature. Laryngeal osteosarcomas may closely simulate sarcomatoid carcinomas, since both entities share clinical, histological and immunohistochemical features. Herein, we report a case of primary laryngeal osteosarcoma in a 55 year old male, focusing on the importance of light microscopic, immunohistochemical and ultrastructural features in accurately establishing the diagnosis. In addition, the current paper provides a review of the English language literature on the subject. Laryngeal osteosarcomas usually carry a dismal prognosis with no general consensus on the most effective mode of therapy. Similarly, the current patient developed bilateral lung metastases 8 months after the initial surgery.