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Intravascular epithelioid hemangioendothelioma (EHE) is a rare endothelial tumor with an intermediate grade of malignancy. We present a case of one woman affected by EHE of the femoral vein. Contrast-enhanced ultrasonography played a role in diagnosing EHE and helped differentiate it from thrombosis. To our knowledge, this is the first reported contrast-enhanced ultrasonography of intravascular EHE in the imaging literature.A 46-year-old woman presented to our hospital due to pain and swelling in her right lower limb since 5 years prior to her presentation.The patient was misdiagnosed as having thrombosis by ultrasound. Contrast-enhanced ultrasonography showed solid lesions with visible blood supply, suggesting angiogenic tumors.The patient was treated by complete surgical removal of the mass and postoperative radiotherapy. Pathological examination confirmed the diagnosis of EHE.During follow-up, there were no signs of local or distant relapse.Intravenous EHE may be misdiagnosed as thrombosis by ultrasound and contrast-enhanced ultrasonography can help make the differential diagnosis.