A case of rhabdomyosarcoma of the tongue in an adult male is reported. The diagnosis of this rare lesion is difficult and should be considered in every growing mass of the head and neck region. It is based on the pathologic examination of tissue samples guided by immunohistological methods. In this case the examination revealed an embryonal type. The tumoral progression is rapidly fatal unless an aggressive therapeutic regimen including surgery and radiotherapy is instituted. In this manner dramatic improvement in survival rate has been achieved in recent years.