Background We add a new rare illustrative case of osteosarcoma of the larynx to the literature. Case presentation The patient (man; 56 years old) first underwent several biopsies, followed in the end by a total laryngectomy. Diagnosis was histological. The patient developed regional and distant metastases and died of disease after 3 months from surgery. Conclusion Osteosarcoma of the larynx is a rare and aggressive tumour with a poor long-term prognosis. The preferred treatment for this tumour is aggressive surgery. To perform a diagnosis we must be in presence of osteoid or of neoplastic osseous tissue directly produced by the neoplastic cells.
