INTRODUCTION Inferior vena cava (IVC) agenesis is one of rare entities of IVC anomalies which presents in young patients with unprovoked deep venous thrombosis (DVT) or unexplained bilateral lower venous insufficiency. We are presenting a case of IVC agenesis which was treated with IVC reconstruction. CASE We describe a case of 28 years old male with painful bilateral lower extremity varicose veins and a history of right lower extremity DVT and was on anticoagulation with warfarin. He was found to have extensive bilateral greater saphenous veins (GSVs) and right femoral vein reflux with patent bilateral deep veins. He was treated with bilateral GSV ablation and microphlebectomies. Six weeks later he presented with acute bilateral iliofemoral DVTs treated with tissue plasminogen activator thrombolysis tPA via bilateral popliteal vein access which helped relieve his leg swelling but he continued to have debilitating venous claudication. A computed tomography (CT scan) demonstrated resolution of DVT but revealed IVC agenesis. He underwent IVC reconstruction with prosthetic graft which helped complete resolution of his chronically debilitating bilateral lower extremity claudication. CONCLUSION In young patients with severe manifestations of lower extremity venous hypertension i.e. edema, varicosity and DVT, central venous anomaly should be considered. Severely symptomatic cases of IVC agenesis can be treated with IVC reconstruction.