Chondrosarcoma is a malignancy of the mesenchymal tissue derived from transformed cells that produce the cartilage matrix. In the neck area, it represents less than 0.5% of malignant tumor pathology. Chondrosarcoma of the hyoid bone is extremely rare, only 20 cases having been published so far (PubMed 2014). We present the case of a 30-year-old patient from the urban area, admitted in the ENT (Ear, Nose & Throat) Emergency Service with inspiratory dyspnea, dysphagia, stomatolalia, with evolutive and progressive clinical history of 2-3 months. Endoscopic examination revealed a pharyngolaryngeal tumor process located in the right vallecula, who by mass effect displaces the above-hyoid epiglottis. CT (computerized tomography) scan described a cervical polycystic tumor aspect, with multiple septae and inside calcifications with a diameter of 3-4 mm. Surgery consisted in removal of the tumor process together with the hyoid bone. Histopathological and especially immunohistochemical examination established the diagnosis of low-grade chondrosarcoma of the hyoid bone. For assessment of the phenotype of the tumor cells, the following immunohistochemical markers were used: p53, Ki67. The patient followed radiochemotherapic oncological treatment and returned for regular follow-ups. There was a positive development with no signs of regional or remote relapse or metastasis for 24 months after surgical treatment. Surgery is the treatment of choice, with complete removal of the tumor, with chemoradiation playing an adjuvant role. Regular tracking of the patient is mandatory.