We report an unusual case of a 40-year-old male patient who experienced painful swelling of the left lower limb that persisted for 1 week. Imaging modalities not only confirmed the diagnosis of acute iliofemoral venous thrombosis and pulmonary embolism (PE), but also an incidental finding of interrupted inferior vena cava (IVC). This congenital anomaly is uncommon but rarely associated with venous thromboembolism (VTE). The azygos continuation was compressed by the descending aorta against the 11th thoracic vertebrae, which was identified as the cause of VTE. He was treated successfully with anticoagulation and compression therapy. The patient was discharged with lifelong oral Rivaroxaban and remained asymptomatic. In the literature, only 9 cases of interrupted IVC-associated PE were identified but none was due to significant venous compression.
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