Laryngeal leiomyosarcoma is rare. The authors report the case of a 48-year-old patient who presented with progressive hoarseness. This symptomatology was associated with a laryngeal tumour. Immunohistopathological study indicated a leiomyosarcoma. Acute dyspnoea resulted in an urgent tracheostomy, followed by total laryngectomy without neck dissection later. In the light of this observation and a literature review, histopathological characteristics, clinical aspects and therapeutic indications will be discussed for this localisation.