A 78-years-old man presented with weight loss, dyspnea, cough and hemoptysis. He was an ex-smoker with a smoking history of 30 pack-years. Imaging studies revealed an endoluminal carinal mass and a tumoral mass arising from the posterior wall of the trachea was observed bronchoscopically. Endoscopic argon plasma coagulation was performed to take biopsies and to regain the airway passage. Recanalisation of the right and left main bronchi and the trachea was achieved together with resection of more than 90% of the tumoural mass. Microscopically, the tumor was composed of fascicular pattern of spindle cells with atypical mitotic figures and nuclear pleomorphism, positively reactive to epithelial membrane antigen supporting a diagnosis of tumour with epithelial differentiation. These findings supported the diagnosis of a spindle cell sarcomatoid carcinoma of trachea. Spindle cell carcinoma cases are reported in many sites, such as head and neck region, larynx, digestive tract, breast, kidney, genital tract, skin and lung, but to our knowledge, not in trachea. Primary malignant tracheal tumours consist mainly of squamous cell carcinoma and adenoid cystic carcinoma and generally have an aggressive course with poor prognosis. We report the first presentation of a spindle cell sarcomatoid carcinoma of trachea together with the clinical course of the patient.