Adenoid cystic carcinoma (ACC) of the trachea is rare, it represents 1% of all respiratory tract cancers. It’s generally considered as a slow- growing, with pronlonged clinical course. Most patients present with dyspnea, and the symptoms often mimic those of asthma or chronic bronchitis Surgical resection is the mainstay of treatment often combined to radiotherapy because of close surgical margins. When surgery isn’t possible, most tumors respond to radiotherapy alone wich often results in long periods of remission We report two cases of primary ACC of trachea: a 49 year old male presented a distal unresectable tracheal ACC treated with chemo-radiotherapy who developed a recurrence and died 7 years after the diagnosis. And a 50 years old female with a proximal tracheal tumor treated by surgical resection and end- to- end anastomosis followed by adjuvant radiotherapy. At 10 months follow-up, our patient shows no evidence of disease with negative histological findings.