A 35-year-old man at our clinic was found to have steatocystoma multiplex manifested by dome-shaped nodules confined to the scalp and forehead. One of four cyst biopsy specimens revealed large lining cells with granular cytoplasm adjacent to typical steatocystoma epithelial lining cells. Neither fibrosis nor inflammatory changes were noted in the surrounding connective tissue. The large granular lining cells had immunohistochemical characteristics of the macrophage/monocyte lineage. This case appears to be an unusual clinical and histological manifestation of steatocystoma multiplex.