Neurofibromatosis type 1（NF1）is rarely accompanied by sphenoid dysplasia and calvarial defects. Patients with orbital wall defects require surgical treatment when they have severe exophthalmos, which is a cause of corneal erosion and loss of vision. We report a 32-year-old woman with NF1 who presented with pulsatile exophthalmos and bulging of the temporal region due to large bone defects in the orbit and around the pterion. Orbital and calvarial reconstruction was performed together with iliac bone grafting and titanium plate insertion. The patient had no recurrence of the symptoms during 10 years of follow-up. In this case, the transcranial approach was helpful to expose the deep orbital lesion and fix the implants. Although bone resorption is problematic, iliac bone grafting is useful for the following reasons: it can be sufficiently harvested to cover both the orbital wall and the calvarial defects, and can be easily shaped to fit into the defects.
