Abstract: Six patients with pigmented neurofibroma were reported.Of the 6 patients,3 were male and 3 were female,with the age varying from 8 to 55 years.All the patients presented with brown patches or plaques ranging from 2 cm × 3 cm to 30 cm × 40 cm in size.Lesions were located on the left lateral neck,trunk and extremities in 3 cases,on the right axillary fossa and left upper arm in 1 case,on the extensor aspect of the left forearm in 1 case,and on the waist and buttocks in 1 case.Three of these patients were complicated by neurofibromatosis type I.Histopathological examination revealed increased pigments in the basal layer,mild hyperkeratinization in the epidermis,and diffuse distribution of tumor cells in the dermis and subcutaneous adipose tissue.The tumor was non-capsulated,and consisted of plenty of spindle cells and a few scattered or clustered melanocytes with melanin granules in the cytoplasm.Immunohistochemical study showed positive staining for S100 and vimentin.None of the patients received treatment.Pigmented neurofibroma is rare in clinic,and should be clinically and histopathologically distinguished from Becker's nevus,congenital melanocytic nevus and pigmented dermatofibrosarcoma protuberans.