Embryonal rhabdomyosarcoma of external ear canal is very rare, even in the pediatric population. We report an embryonal rhabdomyosarcoma of external acoustic canal, occurring in a 12-year-old Chinese girl who presented with left ear canal mass. Wide local excision of the mass was done under local anesthesia. Histopathological examination revealed the diagnosis. She then underwent 20 cycles of radiotherapy. CECT scan post treatment showed clearance of tumor cell. Now, patient is disease free for 5 years. We believe that early diagnosis followed by complete resection of the tumor with clear margin and radiotherapy improves the prognosis of the disease.
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