Neurofibromatosis, now termed neurofibromatosis type I, is known as a congenital and familial disease presenting abnormalities of the skin, nervous system, bones, and soft tissue. We experienced a case of extremely large neurofibromatosis which developed on the orbital and temporal region of a 24-year-old man. The tumor was widely excised including normal skin margin, outer table of cranium, a part of zygoma and maxilla. Bony defect was reconstructed by rib bone graft and secondary cosmetic correction of blepharoptosis was performed using supratarsal fixation in postoperative 6 months.