Epithelioid hemangioendothelioma is a very rare disease affecting big blood vessels,especially veins.There have been very few articles describing the disease.We hereby present the case of a 56-year-old woman presenting with lower limb edema,who was initially being treated for residual thrombus in the common femoral vein but was eventually diagnosed with epithelioid hemangioendothelioma (EHE).The common femoral vein was resected and reconstructed using the exteal jugular vein.No additional therapy was administered.In this article,previous literature about EHE has been reviewed and oncologic principles have been discussed.