Malignant tumors of the trachea are rare. The most frequent histologic type is squamous cell carcinoma. Cartilaginous tumors are rare in the upper airways. There are only four reported cases of chondrosarcomas of the upper third of the trachea. We present a 76-year-old man with an upper third tracheal chondrosarcoma and a review of the literature, focusing on the clinicopathological features.