True renal artery aneurysms are rare. Ruptured aneurysms are even rarer but can have devastating consequences. Renal artery aneurysms most commonly occur in patients with hypertension, atherosclerosis, or fibromuscular dysplasia. Treatment options can range from embolization to nephrectomy. We describe an interesting case of spontaneous accessory renal artery aneurysm rupture in a 44-year-old female with neurofibromatosis type 1 (NF1) who otherwise had no medical problems. She was successfully treated with selective coil embolization, recovered without complications, and maintained preoperative renal function.

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