Kidney and inferior vena cava (IVC) abnormalities with extensive deep vein thrombosis (DVT) is a very rare cause of DVT and has a diverse clinical presentation. Computed tomography (CT) angiography is the gold standard for diagnosis and treatment including thrombectomy, thrombolysis and systemic anticoagulation. We present a rare case of active young healthy male admitted with acute onset of right lower extremity pain and swelling who was found to have extensive DVT on doppler ultrasound. CT abdomen showed extensive clot burden involving right common femoral vein extending into internal and external iliac veins associated with IVC hypoplasia and hypoplastic left kidney. Patient underwent urgent thrombectomy, catheter directed thrombolysis and was discharged home in stable condition on oral anticoagulation.