Nevus lipomatosus superficialis is a rare. disease entity characterized by ectopic dermal fat cells. The lesions are usually either congenital or present by the second decade of life and occur pre-dominantly in the pelvic area. There´are usually no associated abnormalities and familial incidence is not yet reported in English language literature. The authors report a familial case of nevus lipomatosus superficialis with characteristic light and electron microscopic findings.