Tracheal chondrosarcoma are rare diseases, with only 15 cases previously described in the literature between 1959 and 2008. Here we present a rare case of tracheal chondrosarcoma and a review of the literature. Our patient, a 72-year-old man, had progressive throat pain for 2 years before diagnosis. Clinical and imaging investigation revealed a giant tracheal mass that was partially debulked by laser for symptomatic relief. Histologically, the mass was characterized as a low-grade tracheal chondrosarcoma. The patient underwent external-beam radiotherapy (EBRT) and received 60 Gy. At the time this report was written, 7 years after the end of the treatment, the patient was alive and asymptomatic without evidence of locoregional disease. This case and some described in the literature demonstrate the value of EBRT as a single treatment modality in achieving local control. More experience is required to establish the definitive role of radiotherapy in low-grade tracheal chondrosarcoma.