Embryonal rhabdomyosarcoma of the middle ear presenting as sarcoma botryoides. Favorable outcome in a 12-year-old boy.

A 12-year-old boy presented with a history of earache, fever and granuloma of the auditory canal. Biopsy revealed embryonal rhabdomyosarcoma. The patient was treated with chemotherapy and radiation therapy, which led to complete disappearance of the tumor without recurrence after three years. Diagnosis, staging and timing of the treatment plan is discussed for this rare but highly malignant tumor.