Abstract Background Interrupted inferior vena cava (IVC) is a rare developmental defect characterised by azygos continuation following failure of fusion of one or more of the component parts of the embryological IVC. It occurs in approximately one in 5000 of the general population. It is usually an isolated finding and is generally asymptomatic. Case Summary A 42-year-old patient presented for further clarification of syncope with a four-day history of persistent exertional dyspnoea. Computed Tomography (CT) revealed a central pulmonary artery embolism on both sides, with prominent azygos and hemiazygos veins and paravertebral collaterals. For further clarification, a magnetic resonance imaging (MRI) scan was performed, where hypoplasia or stenosis of the inferior suprarenal vena cava was noted. There were collaterals of the azygos and hemiazygos veins. The finding was consistent with the picture of a venous abnormality in the sense of an "interrupted IVC (suprarenal) with azygos continuation." Discussion In the case of an isolated interrupted IVC, the patient is usually asymptomatic, and the vascular anomaly itself does not mandate any treatment. This variation should be considered, especially in young patients with deep venous thrombosis (DVT) when no other reason for thromboembolism is evident. To prevent vascular events such as DVT or pulmonary embolism, lifelong anticoagulation therapy is recommended. An interrupted IVC can also cause procedural difficulties during right heart catheterisation, electrophysiological studies, cardiopulmonary bypass surgery, femoral vein catheter advancement, IVC filter placement, and temporary pacing through the transfemoral route.