Background/Aims: Atypical fibroxanthoma is an uncommon tumor that usually occurs in the skin of the head and neck of the elderly with significant sun exposure. We describe a unique case featuring a rare ocular surface conjunctival tumor (atypical fibroxanthoma) and provide insight on its characteristic clinical features, surgical management, and histology. Methods: A 71-year-old male fisherman with no pertinent ocular history presented to an academic center with a rapidly enlarging bulbar conjunctival mass in the right perilimbal region for the past several months. The patient underwent surgical excisional biopsy with cryotherapy, adjuvant alcohol, and amniotic membrane transplantation. Results: Pathology specimen illustrated an atypical spindle cell tumor with inflammatory cells, increased mitotic activity, cytologic atypia, and positive diffuse staining with CD163 and CD10 consistent with an atypical fibroxanthoma. Conclusion: Atypical fibroxanthoma is an extremely rare ocular surface tumor that may simulate conjunctival or ocular surface squamous neoplasia. While this lesion typically pursues a benign clinical course, it may recur or rarely metastasize. Thus it should be treated aggressively with excisional biopsy, cryotherapy, absolute alcohol, and/or amniotic membrane transplantation.