"Congenital" plexiform neurofibroma of the occipital scalp Case report SAMUEL C. OHAEGBULAM, F.R.C.S. (EDIN), F.R.C.S. (ENG) Neurosurgical Unit, University of Nigeria, Enugu, Nigeria An occipital scalp tumor first noted in a Nigerian girl during the first month of her life gradually increased in size and 13 years later measured 20 x 16 X 17 cm. A skull film revealed a 2 • 4-cm skull defect underneath the tumor over the lambdoid suture. Complete excision of the tumor was achieved although it was adherent to the dura of the transverse sinus. It was histologically confirmed to be plexiform neurofibroma. There were no other signs of yon Recklinghausen's neurofibromatosis. KEY WORDS 9 plexiform neurofibroma 9 scalp 9 congenital skull defect 9 transverse sinus p LEXIFORM neurofibromas have been re- ported to occur at different sites in the body, 1,2 including the limbs, face, trunk, stomach, urinary bladder, and the scalp. The case reported here is of a con- genital plexiform neurofibroma of the oc- cipital scalp that was adherent to the dura of the transverse sinus through a skull defect. Case Report This 13-year-old girl presented with a swelling on the right side of the back of the head. The mother had noticed a small lump in the right occipital scalp during the first month of life. The lump did not upset the baby in any obvious way and normal milestones of development were maintained. The lump had slowly increased in size over 13 years and in the 12 months before admission she had become very conscious of its embarrassing size. She had also complained of dull inter- mittent right-sided headaches. There were no signs suggestive of raised intracranial pressure, and no other complaints. Examination. On examination, we found a healthy looking girl, who had normal mental functions. A large oblong swelling of the scalp measuring 20 X 16 • 17 cm occupied the right parietooccipital area. The margins of the lump were ill-defined in some areas. The surface was irregular with nodular areas that were tender and firm. The overlying skin was normal and was attached to the nodular areas of the lump (Fig. 1). There were no pulsations or bruits. No enlarged lymph nodes were noted in the head and neck. Cranial nerves, motor, sensory, and reflex functions were in- tact and there were no abnormal cerebellar signs. The patient had no other evidence of yon Recklinghausen's disease. J. Neurosurg. / Volume 46 / February, 1977 245