The majority of adenoid cystic carcinoma arise from 3 sites : the major salivary glands, the minor salivary glands of the oral cavity, and the mucus glands of the upper respiratory tract. Primary adenoid cystic carcinoma of the trachea is rare, but is second most common tumor only to squamous cell carcinoma in incidence. Characteristics of adenoid cystic carcinoma are its potential for extending long distance submucosally and for perineural invasion. It grows slowly and may have a prolonged course before diagnosis and after treatment. So, it is difficult to assess the result of treatment and prognosis. Adenoid cystic carcinoma is recognized lately in the course of disease and should be considered in the differential diagnosis of obstructive airway disease. Survival may be prolonged even after recurrence and distant metastasis. We experienced a 72 year old female patient who received total laryngectomy due to adenoid cystic carcinoma of upper trachea. So, this case is discussed and the literatures are reviewed.