| Neurofibromatosis is one of autosomal-dominant neurogenetic diseases with relatively low incidence. The reported case located in whole hips manifesting Cafe-au-lait Spots for 50 years and a elephantiasis neuromatosa for 23 years , its clinical type was defined as neuroflbromatosis type 5. The tumor measured 40 by 40 cm2 in size and was successfully operated with one-stage resection. The flaps both from Cafe-au-lait Spots and normal skin were all alive. Histo-pathological examination showed that the tumor belonged to neurofibroma, mainly consisting of nerve sheath cells. The patient was discharged in good condition and with no recurrence after 4 months follow-up.
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