Extracardiac rhabdomyoma is a rare benign tumor that has a predilection for the skeletal muscle of the head and neck. There have only been 10 cases of vocal cord rhabdomyoma reported in the literature until now. In July 1993, we encountered a 64-year-old male patient who suffered from boarseness for six months. The indirect mirror examination revealed a brownish mass over the left vocal cord. The patient underwent endolaryngomicrosurgery later and some brownish jelly-like material was found in the mass. The pathology revealed rhabdomyoma. The voice was much improved after surgery and no recurrence of tumor was found during the one year and 9 months follow-up period. Vocal cord rhabdomyoma runs a benign course. Beside the clinical manifestation and laryngoscopic examination, the exact diagnosis is based on the histopathology. The treatment of choice is adequate and conservative excision of the tumor. Meanwhile, upper aerodigestive function should be carefully preserved.