Alveolar soft part sarcoma is a rare type of sarcoma that usually affects young adult women. It occurs mostly in the lower extremities, and nearly one quarter of the cases are found in the head and neck region. The most common site of origin in the head and neck region is the tongue followed by the orbit. Herein, we present an unusual case of alveolar soft part sarcoma of the tongue in an 18-year-old woman. The clinical, diagnostic, and therapeutic features of this quite rare entity were discussed.
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