We report a case of pleomorphic adenoma of the trachea in an 8-year-old boy who required emergency surgery for severe respiratory distress. Chest computed tomographic scan and bronchoscopy showed a relatively large mass in distal trachea and right main bronchus and destruction of the lung parenchyma. The patient was subjected to right carinal resection, pneumonectomy, and pericardial patch tracheoplasty for reconstruction of the trachea. Histopathological examination and immunohistochemical staining of tumor specimens were compatible with pleomorphic adenoma. Postoperative follow-up of this patient for a period of 6 months showed satisfactory results with no complications or tumor recurrence. We present not only a very rare benign tracheal tumor in children but also demonstrate successful usage of a free pericardial patch for tracheal reconstruction, although direct anastomosis failed to provide an adequate anastomotic lumen.
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