Cases of a retroperitoneal tumor with double inferior vena cava (IVC) are rarely reported. The present report documents a case of a retroperitoneal lymphoma with double IVC, and discusses its embryological, clinical and radiological significance. In addition, previous cases of a double IVC are reviewed. In the present report, a 52-year-old male patient was hospitalized for a retroperitoneal lymphoma tumor and double IVC. CT urography was performed, whilst a three-dimensional visualization model was also established based on CT data, to reveal a retroperitoneal tumor with double IVC. The present case involved a double IVC with interiliac vein, which was type 2b from the left IVC. The retroperitoneal tumor was identified to be a lymphoma measuring 116x83 mm by percutaneous puncture biopsy. Surgical treatment is generally not recommended for lymphoma. Therefore, this patient was transferred to the Hematology Department for treatment according to the lymphoma management guidelines. The size of the tumor was reduced after chemotherapy during the patient's follow-up. In conclusion, the three-dimensional visualization model can directly and accurately present the anatomical features of the double IVC and its surrounding tissue structure. In addition, variations in the features of IVC can have important clinical significance. It is also important for surgeons, interventional radiologists and clinicians to understand such abnormalities in anatomical features to avoid misdiagnosis and reduce the occurrence of serious intraoperative complications.