Introduction: Rhabdomyosarcoma accounts for up to 60% of childhood soft tissue sarcoma, occurring mostly in the head and neck region but rarely in the ear and temporal areas. Case Report: A 4-year-old boy came to our hospital due to a purulent discharge, bleeding from his left ear. Also seen mass in the left ear along with facial weakness and diplopia. After histopathologic evaluation, it was diagnosed as embryonal rhabdomyosarcoma. Conclusion: Rhabdomyosarcoma should be kept in mind as a differential diagnosis in all children presenting with otitis media with mastoiditis. Biopsy should be considered who are not responding to medications and those worsening condition.
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