Microcystic adnexal carcinoma (MAC) is an uncommon, locally aggressive, malignant cutaneous tumor with pilar and eccrine differentiation. In this article we describe the clinical presentation, pathologic hallmarks, and the use of new adjunct diagnostic devices, to optimize diagnosis, management and treatment options. Ó 2015 The Authors. Production and hosting by Elsevier B.V. on behalf of King Saud University. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).