Persistent left-sided inferior vena cava (VCI) is a rare venous anomaly, its prevalence being estimated at 0.2-0.5%. Thrombotic occlusion of a VCI has been reported in only a few of these cases. We report the case of a 24-year old woman who suffered an acute thrombosis in a left-sided VCI and recurrent pulmonary embolism. After thrombectomy the course was uneventful. The diagnostic approach and the treatment strategy are discussed with reference to the literature.