Rationale:The autosomal dominant condition known as neurofibromatosis type 1 (NF-1) is characterized by café au lait macules and neurofibromatosis. Aneurysms in renal arteries are rare. Renal artery aneurysm (RAA) can be successfully treated with endovascular procedures; however, successful cases in NF-1 adults have not been reported.Patient concerns:Here, we report the case of a 30-year-old female suffering from NF-1. The patient presented to the emergency department with complaints of chronic, poorly controlled hypertension. A left RAA was found om the computed tomography angiography (CTA).Diagnoses:A left renal artery aneurysm was diagnosed using CTA during workup for secondary hypertension.Interventions:Selective angiographym of the left renal artery confirmed a fusiform aneurysm of the distal renal artery. A self-expandable covered stent was placed, and a completion angiogram demonstrated good aneurysm sealing and contrast flow to the left kidney.Outcomes:The patient’s blood pressure improved after the procedure. Her medications were lowered to almost half of their baseline doses, and hydralazine was discontinued. On the follow-up visit after 4 months, the patient reported his home-measured systolic blood pressure to be less than 120 mm Hg. A repeated CTA of the abdomen showed post-left RAA repair with a covered stent and interval improvement of the left kidney.Lessons:RAA caused by NF-1 are manageable and feasible with endovascular intervention.