We report a case of atrophic dermatofibrosarcoma protuberans, an uncommon clinical variant. We discuss molecular and genetic mechanisms of dermatofibrosarcoma protuberans tumorigenesis, review the literature on atrophic dermatofibrosarcoma protuberans, and pose a molecular mechanism for the atrophic variant's phenotype. We also describe using Mohs micrographic excision with CD34 immunostains on the frozen sections to definitively treat the tumor.