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Nutcracker syndrome (NCS) is a rare cause of pelvic venous congestion syndrome and is secondary to either compression of the left renal vein in its normal anatomic position by the superior mesenteric artery and aorta or less commonly when the left renal vein is in a retroaortic position, compressed between the aorta and the spine. We herein present a unique case of NCS in a female patient with a history of chronic pelvic pain and venous congestion. We also review the literature and discuss the diagnostic modalities, differential diagnosi