Synovial sarcoma is a rare mesenchymal neoplasm that is usually located in the limbs. Its occurrence in the head and neck region, and particularly in the larynx, is exceptional, with only 16 cases reported to date. Two histological variants have been described, a biphasic and a monophasic variant. Immunohistochemistry is determinant in the differential diagnosis between synovial sarcoma and other spindle and biphasic neoplasms. The treatment of choice is conservative surgery with tumor-free margins, while radiotherapy is effective in local control of the disease after recurrence. Chemotherapy is indicated for the treatment of distant metastases. We present a case of monophasic synovial sarcoma of the laryngeal ventricle that was successfully treated with CO2 laser excision and we briefly review the literature on the subject.