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. Extraskeletal mesenchymal chondrosarcoma, a rare malignant neoplasm, has high local and metastatic recurrence rate and poor survival. We report here a rare case of iliac vein metastasis in ESMC. Surgical removal of the lesion remains the “gold standard” of treatment for localized tumors. For unresectable, advanced lesions, palliative chemotherapy is recommended.