Neurofibromatosis type 1 (NF1) is an autosomal dominant neurocutaneous disorder with an incidence rate of 1 in 3,500 individuals at birth. The involvement of nasal, sinus or maxillofacial cavities creates airway challenges for anesthesiologists. During the perioperative period, anesthesiologists must be aware of the multisystemic complications of the disorder. Here we report a neglected case of giant NF with deformity of the anterior chest wall (pectus carinatum) and thoracic spine scheduled for tumor resection located at the posterior aspect of the thoracolumbar region. In this case, both anesthesiologists and surgeons faced unique challenges during the perioperative period.