We present a 2 year-old boy with a botryoid type of embryonal nasopharyngeal rhabdomyosarcoma with an unusual outcome. Temporal bone metastasis with brain invasion was detected one year after therapy had been completed. Despite multidisciplinary therapeutic approach, distant metastases occurred in the mastoid and squamous portions of the temporal bone with invasion of the temporal lobe. We review the clinical features of head and neck rhabdomyosarcoma and emphasize the importance of distant tumor metastasis. We believe that this tumor may spread to distant sites by different ways, and its biological course is typically fatal, particularly if recognized in advanced stages.
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