Background: Radical nephrectomy (RN) and caval thrombectomy (CT) for renal cell carcinoma, with extracorporeal circulation (ECC) and deep hypothermic circulatory arrest (DHCA) is a challenging surgical approach. Objective: To assess peri-operative and oncologic outcomes of renal cell carcinoma patients treated with RN and CT, using ECC and DHCA. Design, setting, and participants: We retrospectively evaluated 46 patients who underwent RN and CT using ECC and DHCA. Surgical procedure: After retroperitoneal nodal dissection and RN, a cardiopulmonary bypass was placed and DHCA achieved. A combined approach through the abdomen and the thorax was described. Measurements: Perioperative and long-term survival outcomes were reported. Results and limitations: Median operative time and length of hospital stay were 545. min and 22 d. Overall, 33 patients (72%) did not require any additional interventional or surgical treatment. Thirty-day and 90-d mortality were 11% (5/46) and 15% (7/46). The 1-yr, 2-yr, and 3-yr cancer specific mortality (CSM)-free survival rates were 77%, 62%, and 56%, respectively. After stratification, according to metastatic status at diagnosis, CSM-free survival rates were significantly lower for cM1 patients compared with cM0 patients (1-yr 46% vs 93%, 2-yr 23% vs 81%, 3-yr 23% vs 73%, p <. 0.01). Our study is limited by its retrospective and uncomparative nature. Conclusions: RN with CT using ECC and DHCA is a challenging procedure which requires a dedicated multidisciplinary working team to minimise complications and maximise patients' outcomes. Patient summary: Patients with kidney cancer and a thrombus within the inferior vena cava, which reaches above the diaphragm, can be treated w ith surgery. However, this kind of surgical treatment is challenging and requires a dedicated multidisciplinary team in order to accomplish the task. Radical nephrectomy and caval thrombectomy using extracorporeal circulation and deep hypothermic circulatory arrest is a challenging procedure, which requires a dedicated multidisciplinary working team. We provide evidence that cM0 patients have non-negligible midterm survival. Conversely, in cM1 patients, survival remains invariably poor. © 2017 European Association of Urology.