Primary malignant tracheal tumors are not common and adenoid cystic carcinoma (ACC) of trachea is rare. We report an extremely rare case of ACC of proximal trachea, which was diagnosed in a 42-year-old male who presents with 6-month history of dyspnea. Lateral skiagram of neck, computed tomography, and magnetic resonance imaging revealed a broad-based polypoidal soft tissue mass arising from posterior wall of the proximal trachea. Biopsy confirmed the diagnosis of ACC. The patient was treated by surgical resection followed by radiotherapy and is on regular follow-up. Follow-up at 18 months post-treatment showed no local recurrence or distant metastases. The literature on tracheal ACC is reviewed. Image findings are briefly discussed.