Nevus lipomatosus superficialis is a rare form of benign connective tissue abnormality characterized by the presence of mature ectopic fat tissue in the dermis. Several theories have been proposed for the origin of adipose tissue in nevus lipomatosus superficialis. But the exact pathogenesis remains unclear. Nevus lipomatosus superficialis is classified into two clinical type; a multiple (classic) type and a solitary type. Usually nevus lipomatosus superficialis starts at birth and increase in size in proportion to development. The solitary type is difficult to distinguish from a skin tag and fibrolipoma. Lesion occurs in different areas from the multiple type and the lesion appears in adult life in contrast to classic multiple type. So it may be difficult to diagnosis by clinical features. And histopathological examination is frequently required for diagnosis of solitary type of nevus lipomatosus superficialis, as well as multiple type. A 50-year-old man presented with solitary skin-colored pedunculated mass on Rt. post thigh at birth. In histological examination, the mature fat cells were found to be embedded among the collagen bundles of the dermis, often as high as the papillary dermis. The patient was treated with excision and showed no recurrence for 24 months follow-up. Herein, we report a rare case of nevus lipomatosus superficialis as solitary type.