Alveolar soft part sarcoma is a malignant soft part tumor the etiology of which has not yet been clarified. Histopathological examinations show an alveolar or pseudoglandular pattern. PAS-positive, diastase resistant intraplasmic inclusion bodies are characteristic for this tumor which occurs in the head and neck region; mainly the tongue and the orbita. Primary therapy includes total resection which can later be combined with radiotherapy or chemotherapy. The present study involves a 17 year old patient. We totally removed the tumor and then used radiotherapy. The postoperative examinations over 3 years showed neither local recurrence nor distant metastases.