Adenoid Cystic Carcinoma (ACC) is a rare primary malignancy of trachea. The clinical and pathologic features of ACC of the trachea were initially reported in 1859 by Bill Roth. It represents 0.09% to 0.2% of all thoracic tumors. ACC is characterized by slow growth, and distant metastasis has been reported as late as 25 years after diagnosis. Currently, because of their locally invasive and potentially distant evolution in advanced forms, they are classified as malignant tumors. The clinical presentation of these tracheal localization lesions is often stereotyped. Inspiratory dyspnea is often in the foreground. Surgery is considered as the treatment of choice for ACC, and most articles have focused on the surgical outcome. Radiotherapy (RT) was traditionally used as an adjuvant treatment for controlling microscopic disease or as a salvage treatment for unrespectable disease; however, the exact role of RT remains unclear due to the rarity of such reports. We present here a clinical case of ACC of the lower trachea and different treatment modalities.