We present a surgical case of a rare primary tracheal tumor. In a 44-year-old asymptomatic man, computed tomography (CT), performed as part of health check-up, revealed a tumor measuring 1.5 cm in diameter in the mediastinal trachea. Biopsy failed to yield a definitive diagnosis, but the tumor tended to grow rapidly; therefore, surgery was performed. Five tracheal rings were resected through median sternotomy, followed by interrupted suture with 3-0 absorbable thread. The postoperative course has been favorable with no evidence of recurrence. The pathological diagnosis was leiomyoma. We report this case with literature review.