Rhabdomyosarcoma is relatively seen in the pediatric age group with the head and neck region as the commonest site. To the best of our knowledge, few cases of laryngeal involvement in adult have been described in the literature. Biologically, rhabdomyosarcoma is different from squamous cell carcinoma, which is the commonest tumor of the larynx. A previously healthy non-smoker 77-year-old lady presented to the ENT outpatient with a six weeks history of intermittent alteration of voice quality. She had no history of sore throat, or any symptoms suggesting laryngo-pharyngeal reflux. Examination showed asymmetry of the left arytenoid cartilage and aryepiglottic fold. She subsequently had a direct laryngoscopy and biopsy. Histology and immunohistochemistry examination suggested the diagnosis of mesenchymal neoplasm. Following discussion at MDT she subsequently had a total laryngectomy. Histology confirmed a completely excised laryngeal rhabdomyosarcoma. Rhabdomyosarcoma of larynx in adult is a rare disease. Surgical treatment with or without adjuvant radiotherapy is currently the treatment of choice for this disease.