Tuberous sclerosis with bilateral renal angiomyolipoma and wunderlich's syndrome.

This case describes a 47-year-old female with tuberous sclerosis associated with bilateral giant renal angiomyolipomas. She underwent an initial renal angioembolization in August 2006. After four months, she required emergency nephrectomy due to spontaneous hemorrhage from the angiomyolipoma (Wunderlich's syndrome). Life threatening Wunderlich's syndrome requires emergency nephrectomy, however, renal preservation can be achieved by elective nephron sparing surgery.