OBJECTIVE We report the anesthesia management of a 15 years-old patient with neurofibromatosis type 1, scheduled for resection of a tumor located in the occipitocervical region. In addition, we review the pertaining literature, emphasizing the anesthetic implications of neurofibromatosis manipulation. CASE : A 15-years-old female patient, with Neurofibromatosis type 1 was diagnosed with a large tumor in occipitocervical region suggestive of a plexiform neurofibroma. She presented with cervical instability, difficulty in positioning due to the large cervical mass and other predictors of airway difficulty. Awake intubation was carried out with fiberoptic bronchoscopy after anesthetic block of the airway and remifentanil infusion at low doses (0.05 mcg/kg/min). An inadvertent lesion in the left vertebral artery during the surgical procedure was well controlled by fluid replacement, red blood cell and plasma infusion and norepinephrine. The histopathological report revealed a malignant peripheral nerve sheath tumor originated from a neurofibroma in the craniocervical region. Two months after surgery the patient presented a right crural deficit due to tumor recurrence. CONCLUSION This case report demonstrates the importance of knowing the anesthetic peculiarities of patients affected by Neurofibromatosis type 1 submitted to surgery. Neurofibromatosis is a rare pathology in surgical centers, which requires special attention from the anesthesiologist.