Mohs micrographic surgery is a technique that has been shown to provide superior cure rates in the treatment of many mesenchymal tumors. Here, we report the first case of myofibrosarcoma treated with Mohs micrographic surgery. Myofibrosarcoma is a rare but aggressive tumor that can be difficult to distinguish from other somewhat less aggressive malignancies such as dermatofibrosarcoma protuberans or malignant fibrous histiocytoma. Specific histopathologic criteria are reviewed. We recommend including Mohs micrographic surgery in the armamentarium for the treatment of this rare tumor.