Primary tracheal tumors are relatively rare. Here we report one case of primary adenoid cystic carcinoma of the trachea which was ever misdiagnosed as asthma and hysteria. In this case, the pulmonary function test was normal, and firstly no obvious abnormalities were found in laryngoscopy, bronchoscopy and CT scan of chest. Later a sagittal and coronal reconstruction CT scan of trachea showed a mass situated in the subglottic trachea. Lastly a laryngoscopy was again done after a tracheal incision and showed a small mass in the posterior wall of the subglottic trachea, and tumor ablation was performed. In addition, we reviewed the literature of primary tracheal tumors and summarized the epidemiology, presenting features, available therapeutic options of the disease.