Spontaneous near fatal hemorrhage into neurofibromatosis type 1 lesion in the scalp

Neurofibromatosis type 1 (NF1) (von Recklinghausen's disease) is an autosomal dominant disorder characterized by cafe-au-lait spots, pigmented hamartomas of the iris, and multiple neurofibromas. Patients can present with hemorrhage secondary to trauma or rarely with spontaneous hemorrhage, both of which can be lethal and life-threatening. We report a 14-year-old girl with a rapidly expanding hematoma in a rare presentation of spontaneous bleed into the NF1 lesion in her scalp. Soon after presentation, she went into hemorrhagic shock. Emergency coiling of the left maxillary artery and branches successfully arrested the bleeding, while resuscitation reversed the hemorrhagic shock.