Abstract
The iliac vein is an extremely rare site of metastasis for extraskeletal mesenchymal chondrosarcoma (ESMC). Involvement of the veins usually leads to an extremely dismal prognosis. Here, we report a 50-year-old patient with retroperitoneal mesenchymal chondrosarcoma and initial metastasis to the iliac bone, which further progressed to involve the iliac vein. In this study, we reviewed the major characteristics of ESMC and the previously reported cases, considering the rarity of these tumors.