Adenomyoepitheliomas are biphasic tumors composed of epithelial and myoepithelial components in varying proportions. They are commonly reported in the breast, uncommonly in salivary glands, and rarely in the lungs and vulva. Recent molecular analysis has shown that p27/kip-1 protein may play a role in their development. Primary cutaneous adenomyoepithelioma is an extremely rare tumor with only three well documented cases reported to date. We report a case of primary cutaneous adenomyoepithelioma with histological and immunohistochemical characterization, and discuss their differential diagnosis, biologic behavior and treatment strategies.