Introduction: Rhabdomyosarcoma is the most frequently occurring intrusive soft tissue sarcoma in the pediatric age group. Orbit is the most common location for a pediatric rhabdomyosarcoma, but it can occur in the oral cavity, pharynx, face and neck in the descending order of incidence. Rhabdomyosarcoma in the ear is extremely rare. Case Report: A 5-year-old girl presented to the outpatient department of our tertiary care hospital with complaints of foul smelling, non-blood stained right ear discharge of one-month duration and deviation of angle of mouth to the left side of acute onset. Investigations revealed a diagnosis of embryonal rhabdomyosarcoma. Multimodal therapy was carried out, and the child was rendered disease-free after two years. Conclusion: Embryonal rhabdomyosarcoma of the head and neck mimics chronic otitis media. Early diagnosis is essential to deliver prompt treatment and prevent locoregional spread and metastasis.
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