Atypical fibroxanthoma (AFX) is a rare neoplasm, with a limited number of cases reported in the periocular region. In this case report, we detail a 63-year-old woman who presented with a polypoid, exophytic lesion on her right upper eyelid that had been progressing for a year. The lesion was meticulously excised with security margins and reconstructed using a glabellar flap. Following a thorough microscopic and immunohistochemical analysis, AFX was diagnosed. Despite its sometimes clinical and histological benign appearance, AFX is classified as a malignant neoplasm; however, it carries an excellent prognosis with low metastasis and recurrence rates. Complete excision with safety margins is essential and an adequate post-operative surveillance is recommended. Owing to its rarity, ophthalmologists should remain vigilant and include AFX in their differential diagnosis, as the tumor's benign appearance may lead to misdiagnosis of this malignant entity.
Keywords: Atypical fibroxanthoma; cutaneous tumors; oculoplastic surgery.