A young competitive skier had venous claudication. A stenosis of the left common femoral vein was revealed by means of an examination. Exploration and vein patch angioplasty were performed, and because of both the unusual appearance (focal thickening of vein wall) and the unclear etiology of the lesion, frozen and permanent sections of the wall were obtained. Epithelioid hemangioendothelioma, a rare intravascular sarcoma, was revealed by means of an examination of the permanent sections. Two additional procedures were required to completely excise the epithelioid hemangioendothelioma. We discuss these rare vascular malignancies and include a review of the available literature. Also, oncologic principles important in both the diagnosis and therapy of intravascular sarcomas are discussed.