Primary adenoid cystic carcioma of trachea is rare, with an incidence of only 0.2 per 100,000 persons per year. When all series of the tracheal carcinomas are combined, adenoid cystic carcinoma is the second most common tumor only to squamous cell carcinoma in incidence. Most patients have wheezing or stridor, dyspnea, hemoptysis, and cough as symptoms. Treatment options include surgery alone, radiation therapy alone, or a combination of both. The recommended surgical option is primary tracheal resection and reconstruction. Recently, we experienced a case of adenoid cystic carcinoma in 45 year old female patient who was treated tracheal tumor resection and end-to-end anastomosis of the trachea, so we report this case with the literatures.