Embryonal rhabdomyosarcoma of the middle ear, as previously reported in the literature, is an almost uniformly lethal entity. An unusual case presenting with bone destruction and later demonstrating a metastatic locus has achieved a relatively long‐term NED survival status. Adequate radiotherapy, when combined with both concomitant and then maintenance chemotherapy, may not only control the local tumor but delay, prevent, or treat peripheral macroscopic or microscopic disease.

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