Abstract: A 29-year-old male presented with safe-au-lait-spots and plaque on his neck for two year. Pathologic examination revealed that a well defined unencapsulated lump in dermal, which cinsists of loosely arranged spindle cells with scanty pale cytoplasm and elongated wavy, scattered mast cells within the tumor. Immumohistochemical staining showed S-100 and CD34 were positive.The diagnosis of segmemal neurofibromatosis was made.

Key words: neurofibromatosis, segmemal