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A congenital arteriovenous fistula of the kidney was discovered in a 75-year-old man presenting with hematuria and a silent kidney on I.V.U. The diagnosis had been suggested from the results of pre-operative arteriography examination. Treatment consisted of a total nephrectomy, because of the age of the patient and the hilar site of the "angioma". Reports in the published literature confirm the rare nature of this type of lesion and the difficulties encountered when trying to classify them in precise nosological groups.
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