A 3-year-old boy with inflammatory granulation tissue in the ear canal and typanometric evidence of middle-ear effusion was examined. Subsequently, rhabdomyosarcoma of the middle ear was diagnosed. The insidious, deceptive presentation of this common pediatric tumor in an uncommon location is discussed with indication that early diagnosis and aggressive multimodal antitumor therapy seems to be improving the hitherto gloomy prognosis for these highly lethal middle-ear neoplasms.

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