Cutaneous lipomatous neurofibroma is a rare variant of neurofibroma with a little known pathogenesis; its presence has never been described in Brazil. A 61- year-old woman complaining scalp papules for one year, presented with skin colored to yellowish papules on the scalp. She noted that they were sometimes itchy and occasionally bled.

Dermoscopy showed amorphous material, milky white, with a small poorly delimited yellowish area. Histopathological examination revealed spindle cell proliferation associated with mature adipocytes. Cutaneous lipomatous neurofibroma appears to be an underdiagnosed condition owing to the low index of suspicion and lack of knowledge of its existence. Few cases have been reported in the literature.