Lipomatous metaplasia of the upper dermis is a rare phenomenon scarcely described in the literature which has always been identified in the context of inflammatory or neoplastic processes. Microscopically, it should be distinguished from pseudolipomatosis cutis, dermal lipoma, nevus lipomatosus, and focal dermal hypoplasia (Goltz syndrome). This is the first report of the histologic and ultrasound evaluation of a patient with extensive involvement and the description of the coincidental finding of an anomalous nonseptate subcutaneous tissue.