Synovial sarcomas are malignant tumors of mesenchymal origin, extremely rarely located in the area of the head and neck. Histologically they can be monophasic, biphasic or poorly differentiated with numerous differential diagnostic dilemmas. A 54-year-old male with synovial sarcoma of the carotid space is presented. The patient refused suggested postoperative radiotherapy and, nine months after the primary surgery, local relapse was verified. Following surgical resection of the local relapse, postoperative radiotherapy treatment was utilized. Ten months after the second surgery, secondary deposits in the lungs were radiographically confirmed, and local recurrence was noticed again. Treatment was continued with symptomatic therapy and eleven months later patient died. Synovial sarcomas of the carotid space are extremely rare, with complex surgical approaches and pathohistological differential diagnostic dilemmas. Diagnosis requires determination of the immunophenotype of the tumor cells, whereas therapy requires an aggressive surgical approach and postoperative radiotherapy.