A case of benign cephalic histiocytosis in a 18-month-old male child is reported. Characteristic distribution of the maculo-papular lesions on the cheeks, forehead and earlobes, in addition to light and electron microscopical findings permitted the diagnosis of this rare disorder. Ultrastructurally, worm-like and comma-shaped bodies were seen in the cytoplasm of the histiocytes infiltrating the upper dermis.