The neurofibromatosis is a frequent and polymorphic genetic disorder. The severity is related to the complications. The degeneration of neurofibroma is a very rare complication of neurofibromatosis. In the literature, a few cases of solitary neurofibroma, which turned into a malignant tumor were reported. In our case, we described a very rare clinical case of neurofibrosarcoma in the scalp, and surgical treatment.