Background: Spontaneous arterial bleeding in patients with type 1 neurofibromatosis is uncommon. It can be fatal if not recognized and treated in a timely manner. Objectives: Diagnose and treat an uncommon complication in type 1 neurofibromatosis. Case Report: We report a patient with type 1 neurofibromatosis who bled spontaneously from a pseudoaneurysm arising from a branch of the left thyrocervical artery. The patient was successfully treated with endovascular therapy. Conclusion: Due to the high fragility of involved vessels in patients with type 1 neurofibromatosis, emergent angiography and endovascular therapy should be undertaken without delay, regardless of the presence or absence of contrast extravasation on computed tomography scan.
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