Nutcracker syndrome (NCS) is known as a status of compression of the left renal vein (LRV) between the abdominal aorta and superior mesenteric artery (SMA). We here report a case of NCS in Marfan patients with type B aortic dissection who presented with sudden gross hematuria. Computed tomography revealed the compression of the LRV sandwiched between the SMA and the dilated dissecting abdominal aorta. The compression was released after surgical intervention and the hematuria was promptly recovered. This report highlights that NCS should be considered as a differential diagnosis of unexplained hematuria in patients with the chronic dissecting aorta.