Tracheal chondrosarcomas are rare, with only 14 cases previously documented. We report a case of chondrosarcoma of the trachea and review other published cases. Our patient was a 34-year-old man who began to feel shortness of breath 8 months after initial hemoptysis. Transverse and coronal computed tomography demonstrated a tumor that was obstructing nearly 80% of the tracheal lumen at the thoracic inlet. A bronchoscopic biopsy specimen led to an initial diagnosis of chondroma. Five tracheal rings from the third to the seventh including the tumor were completely resected, and an end-to-end anastomosis was performed. The histological diagnosis of the surgical specimen showed it to be a chondrosarcoma. The patient was alive without recurrence at 6 years 4 months after surgery. Because recurrence after incomplete resection and malignant transformation can occur with chondromas, radical resection of the trachea is recommended for all cartilaginous tumors of the trachea.