Nutcracker syndrome is a rare vascular disorder that involves compression of the left renal vein (LRV), most often at the level of the aortomesenteric angle. In some cases, this compression syndrome arises in the setting of unusual vascular anatomic variants. We describe the case of a 43-year-old woman with a duplicated inferior vena cava who was found to have LRV compression on magnetic resonance angiography and venography with intravascular ultrasound. The patient was successfully treated with concurrent transposition of the LRV and left-sided inferior vena cava, with complete resolution of symptoms. © 2020 The Authors