Rhabdomyosarcoma of the larynx has been very rarely described in pediatric population. There are 3 histological subtypes: embryonal, pleomorphic, and alveolar. With regard to the English literature, we present the first case of alveolar rhabdomyosarcoma of the larynx ever described in a child. This tumor has been diagnosed on an endoscopic biopsy. Thus, a unilateral arytenoidectomy has been performed. This tumor has a poor prognosis. Lymph node metastases were successfully treated by chemotherapy and radiotherapy. Surgery has shifted from radical to conservative combined with adjuvant treatments.
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