Atypical fibroxanthoma (AFX) is a rare skin malignancy generally developed in elderly male patients with actinic damage. Due to its low rate of metastases is considered a benign neoplasm. Metastatic AFX are reported to be less than 6%. Although many risks factors have been described, older age and deep tissue invasion are the most important.

We present the case of a 77 year-old male patient who was referred to the head and neck surgery service for a skin lesion in the scalp. Initially, he complained of a painless, ulcerated and elevated nodule, of one-year evolution. A full thickness resection with oncologic margin was performed and AFX was diagnosed after final immunohistochemistry (IHC). After a two-year follow up, the patient presented a recurrence with a ulcerated, firm erythematous nodule associated with enlarged neck and supraclavicular lymph nodes. A CT scan was performed evidencing bone and lung secundarism as well as cervical, supraclavicular and mediastinic adenopathies. A fine needle aspiration (FNA) biopsy was performed confirming AFX as primary lesion. The oncological board committee recommended surgical and adjuvant treatment. However, the patient worsened his condition, and both the family and the attending team finally decided palliative care.

AFX is regarded to be a benign skin tumor developed in sun - damaged skin, mainly in male senior patients, most often in head and neck region and with a very low metastatic potential.

The main risks factors associated to this tumor are sun exposure, previous traumas and history of radiation. It is usually presented as an elevated reddish painless solid nodule limited to the dermis; it might have rapid growth.

Some authors consider AFX as a superficial variant of malignant fibrous histiocytoma (MFH), which is both histologically and immunohistochemically indistinguishable.

Differential diagnosis includes: melanoma, MFH, dermatofibrosarcoma and others spindle-cell neoplasms. Histological description of the tissue includes atypical spindled cells and pleomorphic cells. The IHC analysis might help determine the final diagnoses, it is negative for S100, CD23 and epithelial membrane antigen (EMA) as well as MELAN A, cytokeratin and desmin, among others. However, there are no specific stains to identify AFX; it is generally positive for vimentin, CD 10, CD 99 and CD 68.

Surgery has been described as the therapy of choice. One-centimeter margin has been proposed although no studies support the indication.

The reported rate of recurrence and metastases is around 14% and less than 6%, respectively. Vascular and perineural invasion, older age, insufficient margins of resection, necrosis and deep tissue invasion have been described as risk factors for both recurrence and distal metastases. The median time reported for these events to arise is between 6 to 18 months.  Most patients who developed extended disease have poor prognosis.

Recurrence and systemic metastases are a rare entity after AFX resection. They appearance during follow up is related to poor prognosis a fatal outcomes.