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Aggressive angiomyxoma is a mesenchymal tumor arising from connective tissues with a predilection to the female pelvic soft parts. Cervico-facial localization remains rare .We report here the first case of aggressive angiomyxoma of the external ear of a 7 year old patient, which was treated successfully by surgical excision. The clinical and pathologic features and radiological aspects of this tumor are discussed.

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