Squamoid eccrine ductal carcinoma (SEDC) is a rare and under-recognized primary cutaneous tumor with a high risk for local recurrence and metastasis. The tumor has a biphasic histologic appearance consisting of a superficial portion indistinguishable from squamous cell carcinoma (SCC) and a deeper component demonstrating eccrine ductal differentiation. Because of superficial sampling, SEDC often is misdiagnosed as SCC during the initial biopsy. The diagnosis usually is made during complete excision when deeper tissue is sampled. Confirmation of the diagnosis can be achieved by immunohistochemical positivity for carcinoembryonic antigen (CEA), epithelial membrane antigen (EMA), cytokeratin (CK) 5/6, and p63. In this article, we review the clinical and histologic details of 5 patients with SEDC who underwent successful treatment with Mohs micrographic surgery (MMS) at a single institution between November 2018 and May 2020. We also review the histologic patterns that helped distinguish SEDC from SCC upon complete excision. Our findings support the use of MMS as the treatment of choice for SEDC, given that all of the patients we reviewed required more than 1 Mohs stage for complete tumor clearance, and none demonstrated evidence of recurrence or metastasis after a mean follow-up period of 11 months.