Giant nevus lipomatosus superficialis with multiple folliculosebaceous cystic hamartomas and dermoid cysts

Nevus lipomatosus superficialis (NLS) is a rare benign condition characterized by papules and nodules usually in the pelvic and sacral areas, with ectopic mature adipose tissue reaching the superficial dermis. We report the case of a 47-year-old female with large NLS in the sacral region that had been present since birth and, after an asymptomatic course for most of the time, became associated with episodes of mild pain that prompted the patient to look for medical help. After a complete resection of the cerebriform plaque, measuring 23 cm in diameter, histopathological examination revealed typical NLS architecture in as yet undescribed association with multiple folliculosebaceous cystic hamartomas (FCHs) and dermoid cysts.