Lipedematous scalp (LS) is a rare condition of unknown etiology characterized by a thick, boggy scalp that occurs mostly in adult black females. The main pathologic finding consists of increased thickness of scalp resulting from thickening of the subcutaneous fat layer. Nevus lipomatosus superficialis (NLS) is an uncommon hamartomatous skin tumor characterized by soft, yellowish papules, or cerebriform plaques, usually localized on the buttock or thigh. Here, we report a 46-year-old woman who has NLS localized on the occipital area, accompanied by LS just beneath and peripheral area of this plaque. The diagnoses of NLS and LS were established by histopathological examination. In addition, the increase in subcutaneous fat layer was demonstrated using magnetic resonance imaging. The presented patient is the first case showing colocalization of NLS and LS.