The improper embryologic development of the inferior vena cava (IVC) is a rare congenital defect that affects about 4% of the population. IVC agenesis is one of the less common variations, with an estimated prevalence ranging from 0.005% to 1%. We present a rare instance of IVC agenesis, bilateral double renal veins, and left circumaortic renal vein that manifested as lower extremities deep vein thrombosis. While anomalies of the IVC and renal veins are exceedingly rare, they should be considered during retroperitoneal procedures to avoid major consequences, particularly venous bleeding caused by incorrect damage. Anatomical variance of the IVC and renal veins is critical for procedures such as ureteral surgery, sympathectomy, radical nephrectomy, and organ transplantation. The clinical significance of these aberrations stems from the possibility of mistakes during imaging. They play a significant role in the diagnosis and treatment of venous thromboembolic disease.