Rhabdomyosarcoma is a rare tumour in the middle ear and mastoid cavity in children and the diagnosis is difficult. Repeated histological examination may be essential to confirm the diagnosis. We report a 6 year old boy with a left aural polyp, otorrhoea and facial nerve palsy who was initially thought to have otitis media and mastoiditis. He had polypectomy and the tissue taken for histopathology suggested an inflammatory condition. Subsequently he had mastoidectomy. Tissue taken during mastoidectomy was however reported as rhabdomyosarcoma. The child developed a cerebral abscess and eventually succumbed. A literature review of the disease, radiological findings, immunohistochemical features and treatment options is described.