Background:Squamoid eccrine ductal carcinoma (SEDC) represents a subtype of eccrine carcinomas that are diagnostically challenging for both clinicians and dermatopathologists.Objective:To provide an updated review of SEDC and examine patient outcomes with different treatment modalities.Methods:A review of Ovid MEDLINE was performed to review the English language medical literature of SEDC.Results:A comprehensive review of clinical presentation, histologic findings, rates of lymphovascular and metastatic disease, treatment modalities and recurrence rates are reviewed.Limitations:There is a limitation on available data because of the rare incidence.Conclusions:SEDC is a rare adnexal neoplasm with a relatively high rate of local recurrence, lymphovascular invasion, perineural invasion, and metastases. Clinicians should be aware of this entity as close follow-up is essential to detect recurrence and metastatic disease. Mohs micrographic surgery seems to result in superior patient outcomes.