Atypical fibroxanthomaAtypical fibroxanthoma (AFX) is an uncommon neoplasmNeoplasm that typically involves sun-damaged areas of the head and neck; it is seen most frequently in elderly white men. UV radiationRadiation has been found to cause DNA damageDamages in dermal fibroblast- or myofibroblast-like cells and has been associated with the development of AFX. AFX typically presents as a rapidly growing, red, often ulcerated nodule on the ear, face, or scalp. AFX can resemble several other neoplasmsNeoplasm, both clinically and histologically. Therefore, histologic examination, along with use of immunohistochemical stainsImmunohistochemical stains, is essential in the diagnosis of AFX. Once a diagnosis of AFX is made, the treatmentTreatment of choice is complete surgical removal. TreatmentTreatment with Mohs micrographic surgeryMohs micrographic surgery (MMS) has been found to be superior to treatmentTreatment with wide local excisionWide local excision (WLE). MMS has the advantages of allowing for tissue conservation, which is especially important on the head and neck, and of achieving total microscopic margin control. Local recurrence, metastasis to regional lymph nodes, and distant metastasis have been reported; therefore, regular, long-term follow-up is recommended for at least the first 2 years after diagnosis of AFX.