We present a case of granular cell atypical fibroxanthoma of the scalp. The neoplasm occurred as a tender nodule on the frontal scalp of an 82-year-old Caucasian man. Histology demonstrated a diffuse proliferation of atypical epithelioid cells with abundant granular cytoplasm. Many of the cells had large irregular nuclei and atypical mitotic figures were present. Immunohistochemical stains were positive for CD68 and procollagen 1 and negative for cytokeratin, HMB-45 and S-100 protein. The granular cell phenotype has been observed in other cutaneous neoplasms including granular cell tumors, dermatofibromas, dermatofibrosarcoma protuberans, fibrous papules, basal cell carcinomas, leiomyosarcomas, angiosarcomas and primitive polypoid granular cell tumors. We discuss the differential diagnosis and review the previously reported cases of this rare variant of atypical fibroxanthoma.