Nevus lipomatosus superficialis (NLS) is a hamartoma of adipose tissue, rarely reported in the past 100 years. We treated one case, and we conducted a systematic review of the literature. A 41-year-old man presented with a cutaneous multinodular lesion in the posterior region near the right auricle. The lesion was excised and examined histopathologically. To review the literature, we searched PubMed with the keyword “NLS.” The search was limited to articles written in English and whose full text was available. We analyzed the following data: year of report, nation of corresponding author, sex of patient, age at onset, duration of disease, location of lesion, type of lesion, associated symptoms, pathological findings, and treatment. Of 158 relevant articles in PubMed, 112 fulfilled our inclusion criteria; these referred to a total of 149 cases (cases with insufficient clinical information were excluded). In rare cases, the diagnosis of NLS was confirmed when the lesion coexisted with sebaceous trichofolliculoma and Demodex infestation. Clinical awareness of NLS has increased recently. NLS is an indolent and asymptomatic benign neoplasm that may exhibit malignant behavior in terms of huge lesion size and specific anatomical location. Early detection and curative treatment should be promoted.