In order to recognize and summarize all data present in literature, we also performed a comprehensive review using the following keywords: “Endocrine mucin-producing sweat gland carcinoma” and/or “EMPSGC” and/or “skin” AND “cutaneous neoplasms”, on the PubMed, Scopus, Web of Sciences (WoS), and Google Scholar databases with particular attention to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. This evidence concerns the gene MUC5AC and sweat gland carcinoma.