Mesenchymal chondrosarcoma is a rare, aggressive, malignant neoplasm, which arises from extraskeletal sites in 30–40 per cent of cases. It is extremely rare in children. We present a novel case of childhood mesenchymal chondsarcoma arising from the vagus nerve in the neck, resulting in paralysis of the right vocal fold. The clinicopathologic features and management of this case are described along with a brief discussion on the aetiology of vocal fold paralysis in this age group. Current literature on extraskeletal presentation of mesenchymal chondrosarcoma is reviewed.