Endocrine Mucin-Producing Sweat Gland Carcinoma (EMPSGC) is a rare, low-grade, malignant tumor derived from sweat glands, arising preferentially in skin from the periorbital region. EMPSGC tumors are more frequently found in elderly female patients. To date, only around 70 cases have been reported in the literature [1-3]. Therefore, thorough immunohistochemical characterization of EMPSGC tumors is of pivotal importance to confirm its diagnosis [4-6].