BACKGROUND We describe a 53-year-old man who presented with a painless neck mass and underwent a resection that identified the tumor as a low-grade chondrosarcoma of the hyoid bone. We reviewed the literature for diagnosis and management options of this exceptionally rare diagnosis. METHODS We conducted a search of the published medical literature before September 2012 in MEDLINE and PubMed using the terms "chondrosarcoma" and "hyoid." The search led to 19 case reports. We then searched for recent advances in diagnosis and treatment. RESULTS Chondrosarcomas of the hyoid bone present as slow-growing, painless masses of the neck. CT and MRI are useful for imaging, but definitive diagnosis is made by biopsy. CONCLUSIONS The preferred treatment is complete surgical removal, whereas radiation is used as adjuvant therapy. Positive surgical margin is a significant risk factor for recurrence. Long-term follow-up is recommended because of recurrences many years later.