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A 36-year-old young woman with a medical history of recurrent pulmonary embolism and chronic pelvic pain was admitted to our hospital. Contrast-enhanced imaging techniques revealed a large left renal vein aneurysm with a coexisting vascular mass. The patient was operated on electively, and the left kidney was autotransplanted to the right ileac fossa following the ex vivo resection of the vascular mass and the left renal vein aneurysm. Herein, we report an unusual coexistence of a vascular mass and recurrent pulmonary embolism treated successfully with our surgical treatment strategy.