Purpose: Resection and reconstruction of the inferior vena cava (IVC) is an unusual and challenging procedure. Malignant disease invading or obstructing IVC is the most frequent entity, while nonmalignant is rare and differential diagnostic is difficult. Methods: We report two cases required resection and reconstruction of IVC. Results: A 31-year-old man with left lower extremity paresthesias. His work-up revealed a large (9x3 cm) mass at the right renal hilum involving the IVC and right renal vein (RV) with complete thrombosis of bilateral external and common iliac veins (CIV), as well as the infrarenal IVC. The tumor contacted the aorta in greater than 90 degrees, as well as the third portion of the duodenum. Surgical intervention: through a midline laparotomy, we attained complete control of the IVC (above and below) as well as left RV and bilateral CIV. The IVC was transected at its origin. After thrombectomy of both iliac veins, no relevant venous flow was observed, therefore we sutured the lower IVC stump. We then divided the left RV and the IVC above the RV level, extracting the tumor en-bloc with the IVC and the right kidney. Finally, we anastomosed the left RV to a “cone” cavoplasty of the remaining IVC stump. Histologically, it was a large (11x4.5 cm) benign mesenchymal tumor involving the right renal hilum, with the diagnosis of calcifying fibrous tumor, a rare benign tumor. During follow-up after 6 years patient develops post-thrombotic syndrome of the lower limbs and subcutaneous venous collateral circulation, but without any evidence of tumor recurrence. On the other hand, we present a 39-years-old male with history of left nephrectomy after inferior vena cava and left RV thrombosis when he was one month old. He came for outpatient consultation due to recurrent deep vein thrombosis and thrombophlebitis. CT scan revealed absence of the infrarenal IVC with prominent collateral veins in multiple pathways. Due to unfavorable response to medical treatment, a surgical approach was offered. IVC reconstruction was performed with cadaveric vein graft, from the remaining iliac veins to the junction of the IVC with the right RV. At 2 years of follow up, the patient presented partial clinical improvement, nevertheless, an MRI scan showed thrombosis of the graft. Conclusion: Transplant and Hepato-Pancreato-Biliary surgeons are familiarized with IVC approach, but surgical treatment is usually difficult and may require a major vascular reconstruction.