Primary adenocarcinoma of the trachea is extremely rare and a standard treatment does not exist due to nonavailability of evidence-based randomized control studies. This paper reports the case of a 60-year-old male, who presented with cough and occasional respiratory distress. Bronchoscopic examination and a computed tomography scan revealed a soft tissue mass in the trachea arising from the posterior tracheal wall. Cytological examination and immunochemistry confirmed primary adenocarcinoma of the trachea. Excision of the tumor followed by three-dimensional conformal radiation therapy was performed, and a dose of 56 Gy was delivered to the primary site. Two and a half years after treatment, the patient has no clinical or radiological evidence of the disease, and no late complication has occurred.

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