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We present a case of spontaneous renal artery dissection (SRAD) in a 28-year-old female with history of neurofibromatosis type I (NF-1) treated successfully with endovascular stenting. The clinical presentation, diagnostic testing, and treatment options are discussed. An endovascular approach with stenting was successfully performed after failure of medical treatment with subcutaneous low molecular weight heparin. Patient’s blood pressure and symptoms improved significantly. This may be the first reported case of SRAD in a patient with NF-1 successfully treated with endovascular stenting.