Primary malignant tracheal tumors are not common and adenoid cystic carcinoma (ACC) of trachea is very rare. The diagnosis is often delayed due to the atypical symptoms. We report an extremely rare case of ACC of proximal trachea, in a 55-year-old female who presented with a 12 month history of progressive dyspnea. Laryngoscopy and computed tomography revealed a broad-based polypoidal mass arising from posterior wall of the proximal trachea. Biopsy confirmed the diagnosis of ACC. The patient underwent a complete surgical resection and post operative radiotherapy. Six months follow-up of the patient did not reveal local recurrence or distant metastases. The literature of tracheal ACC is reviewed.