A case of rare epithelioid hemangioendothelioma with multiple foot and ankle lytic lesions in a 41-year-old male is reported. The patient presented to our hospital after having received treatment elsewhere and developing a local postoperative infection. After thorough investigations and establishing the diagnosis, we initially treated the local infection and highlighted the potential risk of malignancy. Finally, respecting the patient's wishes, he was treated with consideration mostly of the pending foot and ankle fractures rather than the risk of malignancy. At 9.5 years postoperatively, the patient was clinically well and asymptomatic, without clinical, laboratory, or radiologic signs of malignancy, and the previous infection might have even played a remote role in that outcome. A review of the published data regarding the treatment of this unpredictable neoplasm is also presented.