Nevus lipomatosus superficialis is a relatively rare, benign hamartoma composed of mature fat. Nevus lipomatosus superficialis presents at birth or later in childhood as asymptomatic, soft or rubbery papules, nodules, and plaques with a polypoid or cerebriform appearance, most commonly on the buttocks or upper thighs. Cases of presentation in adulthood have been described, and in these later onset cases, lesions are often solitary. Extremely rare cases of generalized nevus lipomatosus have also been described. There is no known sex predilection. The pathogenesis is unknown.