IntroductionA chondrosarcoma originating from the hyoid bone is very rare. Here, we describe a case of low-grade chondrosarcoma of hyoid origin and discuss its preoperative imaging features, including those on positron emission tomography-computed tomography, and its recurrence rate.Case presentationA 42-year-old Japanese man noticed a mass in the right submandibular region of his neck. A hard 3.0 × 2.8 cm tumor was noted on the right side of his hyoid bone. The mass was immobile and moved with deglutition.ConclusionEven though radiographic studies, including positron emission tomography-computed tomography, were inconclusive, the cartilaginous tumor was surgically removed en bloc, and the tumor was diagnosed based on the results of pathological investigations. Close follow-up is recommended in such cases due to the potential for recurrences, because local recurrence occurred in 50% of the reported cases of grade one chondrosarcomas.