Primary cutaneous mucinous carcinoma (PCMC) is an exceedingly rare, low-grade tumor that histologically resembles mucinous carcinoma from other primary sites, such as the breast, gastrointestinal tract, and lungs.The purpose of this article was to review the current literature on PCMC as it relates to epidemiology, clinical presentation, histopathology, immunohistochemistry, treatment, and prognosis.An extensive literature review was conducted using PubMed and Ovid MEDLINE to identify articles related to PCMC.Several hundred cases have been reported in the medical literature, and surgical resection, whenever feasible, is the standard of care.The diagnosis of primary cutaneous mucinous carcinoma is one of exclusion, requiring a metastatic work-up to rule out distant primary. Mohs micrographic surgery is a tissue sparing technique that allows complete margin control of these rare neoplasia.