Purpose <p>Patients with metastatic chondrosarcoma at presentation have a poor prognosis. Owing to the lack of sensitivity to chemotherapy and radiotherapy, surgical resection is the mainstay of treatment of localized chondrosarcoma. The aim of this study was to investigate whether surgical resection of the primary site was associated with improved survival in patients with metastatic chondrosarcoma at presentation.</p> Methods <p>Sixty-two patients with distant metastases at initial diagnosis who were pathologically diagnosed with grade 2 or 3 conventional chondrosarcoma, dedifferentiated chondrosarcoma, or mesenchymal chondrosarcoma between 2001 and 2022 and registered in the Japanese National Bone and Soft Tissue Tumour Registry database were analysed retrospectively. Propensity score matching was performed to correct for differences in background characteristics, including the number of organs affected by metastases, performance of metastasectomy, and performance of chemotherapy, between patients who underwent surgical resection of the primary site (<i>N</i> = 31) and those who did not (<i>N</i> = 31).</p> Results <p>The median disease-specific survival was six months for the whole cohort, 12 months for the patients who underwent surgical resection of the primary site, and two months for the patients who did not. Overall, 37 patients (59.7%) died of the disease. Univariate analysis showed a better disease-specific survival for patients who underwent surgical resection of the primary site compared with patients who did not (2-year disease-specific survival 51.0% [95% CI: 32.3–69.4] vs. 19.3% [95% CI: 6.9–43.7]; <i>P</i> = 0.005).</p> Conclusion <p>Surgical resection of the primary site may increase the survival of patients with metastatic chondrosarcoma at presentation.</p>