Epithelioid hemangioendothelioma is a rare vascular tumor with unpredictable behavior. It often presents as liver or lung nodules and is refractory to conventional chemotherapy. Surgical resection and/or liver transplantation have been the mainstay of therapy, but the results are mixed and unsatisfactory. Although the etiology of this malignancy is unknown, an increased incidence in women has been noted, and an association with exposure to vinyl chloride is emerging as a possible risk factor. This review proposes polyurethane/silicone breast implants as a potential cause of the condition and highlights the dramatic response to interferon (IFN)-alpha in a woman, so exposed, with widespread disease in the liver and lungs. The implications for other women who have had this type of implant could be significant, and the early use of IFN-alpha may be optimal.