Congenital renal arteriovenous malformations (AVM) are rare, with approximately 50 cases reported in the English literature. Typically they are small (1-2 cm) and the majority present with gross hematuria. The authors report a case of a young woman with a 6 cm congenital renal AVM diagnosed during her first pregnancy when she presented with signs of hypertension. Hematuria and rupture of the AVM were not part of her clinical course and a subsequent partial nephrectomy was curative. A review of the English literature of congenital AVMs is presented.
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