Chronic lymphedema is rarely complicated by an angiosarcoma. Angiosarcoma superimposed on chronic lymphedema (Stewart-Treves syndrome) is usually seen post breast cancer surgery accompanied by lymph node resection of the axilla. This is a case report of a 59-year-old male patient with elephantiasis that developed an angiosarcoma of the lower leg. He died a month after the diagnostic biopsy was obtained. This is a rare multifocal tumor in a male with an unusual lower leg location. We reviewed the literature and the need to differentiate this often deadly lesion from a Kaposi's sarcoma.