Iliac vein compression syndrome (IVCS) is defined as extrinsic compression of the left common iliac vein (LCIV) between the overlying right common iliac artery and the lumbar vertebra. The most severe complication is phlegmasia cerulea dolens (PCD), a medical emergency that requires quick intervention to prevent irreversible limb ischemia. This article reports the case of a patient with PCD as the first manifestation of IVCS. The treatment included embolectomy and fasciotomy. Bilateral femoral iliac axis phlebography and cavography were performed 48 hours after the procedure. The IVCS was identified, and balloon predilatation of the lesions followed by implantation of self-expanding stents from the confluence of the LCIV with the inferior vena cava to the middle portion of the left external iliac vein was performed. Postprocedure phlebography demonstrated satisfactory final results, and a 12-month follow-up image showed patent stents and minimal intimal hyperplasia.