Primary neoplasms of the trachea are extremely rare. The diagnosis usually depends on computed tomography (CT) scan. We report a 50-year-old woman who suffered from chronic productive cough for 1 year. Roentgenograms and chest CT showed no abnormal finding. Bronchoscopy showed diffuse circumferential papilloma-like lesions at the trachea. Endobronchial ultrasound showed submucosal invasion of the trachea. The pathology of the endotracheal biopsy showed squamous cell carcinoma. She received photodynamic therapy as first-line treatment with a good response and tumor regression.