Lipomatous neurofibroma is a recently described, very rare variant of neurofibroma. It shows a well-circumscribed nodule consisting of fascicles or whorls of spindle cells with an extensive lipomatous component. These fat cells are considered to be an integral part of this tumor, not a metaplastic or degenerative process. It may be due to the differentiation of local stem cells of neural crest. Herein we report a case of lipomatous neurofibroma, the rare pathological variant of neurofibroma with extensive mature fat cells.