A 70-year-old man complained of right leg swelling due to right iliac vein stenosis. No mass was identified around the stenotic site, and the vessel wall had not become thickened. Self-expandable stents were positioned at the stenotic site. About two years later, chest CT revealed lung nodules. Pathology showed sarcoma. A mass that was considered to be the primary lesion was found around the stent in the right iliac vein. Although sarcoma of the iliac vein is very rare, it should be considered in the differential diagnosis of iliac vein stenosis, even if there are no suspicious findings from image studies.