A case of congenital A-V fistula in the breast of a young woman is presented. The chief complaint was murmur when lying down, especially in the prone position, a palpable lump and the presence of varicose-like veins in the breast. Arteriography revealed an A-V fistula in the left breast. The arterial supply originated from the ipsilateral internal mammary vein and superior thoracic artery and drainage was towards the axillary vein and, to a minor degree, towards the internal mammary vein. The lesion was corrected surgically. Six months later there was no evidence of recurrence.
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