Primary tumor of the trachea is rare. Although most tumors in adults are malignant, various benign tumors might occur. However, pleomorphic adenoma of the trachea is extremely rare. So far, few cases have been reported in medical literature. We report a case of a 38-year-old man with 6 months history of dyspnea and wheezing; the asthma was the initial diagnosis formulated by his family doctor. However, the symptoms were not improved by asthma treatment. The patient was admitted to the emergency department for respiratory distress, and underwent nasal continuous positive airway pressure. Immediately, after an urgent chest computed tomography (CT) was done and revealed an obstructive tumor of the distal trachea which was homogeneous with a smooth and regular surface suggesting its benignity. The patient was operated urgently, the histological examination of the surgical specimen revealed pleomorphic adenoma. We present this case to inform colleagues that pleomorphic adenoma must be considered in the differential diagnosis of tracheal tumors, especially if we have an appearance on CT suggesting the benignity. Surgery is the treatment of choice of this tumor with the lowest recurrence probability.