Clinical Practice and Cases in Emergency Medicine

Neurofibromatosis (NF) is a common autosomal dominant disorder that can be subdivided into type 1, type 2, and schwannomatosis. Patients with NF1 typically develop café-au-lait spots, scoliosis, and benign neurofibromas. In addition, NF1 predisposes to vascular complications including stenosis, arterial ectasia, and aneurysms. Here, we report the case of an otherwise healthy 32-year-old man who developed a fatal tension hemothorax due to vertebral artery aneurysm rupture. Based on the available literature, we discuss the presentation, workup, and available therapeutic approaches to this complication of neurofibromatosis.