Key Clinical Message : Congenital absence of Inferior Vena cava is a rare anomaly . As the case for other congenital anomalies , it should be suspected at all ages in any patient who presents with recurrent complications of venous stagnation such as PE and recurrent DVTs . Key Clinical findings are : recurrent DVT and/or PE and Stigmata of Venous engorgement as a result of development/enlargement of collateral veins . 
Background : Congenital absence of Inferior Vena Cava ( CAIVC ) is a rare anomaly in which the lower-body venous return is impaired . There is usually engorgement of other veins especially Azygos and Hemiazygos systems.
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Case summary : a 47 year-old man successfully treated for PE . Later discovered to have left sided venous engorgement in the abdomen and left lower limb . He was later found to have congenital absence of inferior vena cava and was prepared for further investigations including venography and coagulation profile .