Rhabdomyosarcoma (RMS) is malignant tumor and develops from the embryonal mesenchyme which give rise to striated muscle. Laryngeal localization of RMS is extremely rare. Cel- lular differentiation may be very different in this tumor and attempts have been made to correlated the morphological patterns with clinical course. A 39-year-old male suffered from hoarseness and dyspnea, visited to the department of inter- nal medicine, Wallace Memorial Baptist Hospital. Local excisional biopsy under indirect laryn- goscopy was done. A biopsy with review and compare it with previous reports.