A case of left facial elephantiasis neurofibromatosa was treated with near-total excision and skin graft. Results were favorable. Repositioning of canthal ligaments, preserving part of relatively normal masseter muscle and primary nasal reconstruction with forehead flap were performed. The skin graft over the myxomatous muscle was successful. Troublesome bleeding was avoided or controlled by incising the normal skin, subperiosteal dissection, hypotensive anesthesia, and chromic catgut suture ligature.