Epithelioid hemangioendothelioma (EHE) is an extremely rare vascular tumor of intermediate or “borderline” malignancy. Generally it develops from the soft tissue, the lung or the liver and occasionally is known to metastasize. We report a case of EHE of a remnant and atrophic femoral vein associated with a persistent sciatic vein (PSV), that developed in a 69-year-old woman. She complained of a solitary painful mass on the right groin. She underwent complete resection of the tumor and the segments of the iliac-femoral artery and vein involved. The defect of the iliac-femoral artery was reconstructed with a prosthetic graft, but the iliac-femoral vein was not reconstructed because of the patency of the PSV. The pathological examination revealed EHE with low mitoses and bone formation. Radiation and chemotherapy do not improve survival or prevent recurrence, so that the need for complete excision is emphasized. To the best of our knowledge, this is the first case report of EHE arising from the femoral vein in such an unique situation in Japan.