We present a case of inflammatory myofibroblastic tumour (IMT) of the trachea arising in a young woman. The patient presented with acute upper airway obstruction, dyspnoea and cough. At videobronchoscopy, the lesion appeared as a smooth, pink, polipoid mass of 1,5 cm obstructing the 70% of the tracheal lumen. The mass was endoscopically removed. Histopathologic examination revealed a lesion composed of spindle shaped cells admixed with a prominent inflammatory infiltrate, which included hystiocytes, plasma cells and lymphocytes. Immunohistochemical studies revealed that the spindle cells were reactive with anti-vimentin, while cytokeratin, S-100, smooth muscle actin, CD21, CD23, CD34 and CD31 were all consistently negative. The proliferative index, shown by Ki67 antibody, was about 7% . Two years after diagnosis , the patient is well without evidence of disease. IMT of the respiratory tract occurs more frequently in the lung. To the best of our knowledge there are only 2 previously reported cases of tracheal IMT. This type of lesion, has to be differentiated from malignant neoplasms such as spindle cell carcinoma and fibrosarcoma