Nutcracker syndrome (NCS), the left renal vein hypertension due to compression between aorta and superior mesenteric artery, may present with hematuria, flank pain and proteinuria. We report a 6-year-old girl with episodic macroscopic hematuria who was diagnosed as NCS with Doppler ultrasound and 3D computerized tomography angiography. She was managed conservatively with spontaneous resolution of macroscopic hematuria. With this case we would like to emphasize that in children with hematuria and orthostatic proteinuria without an apparent cause, a great index of suspicion and appropriate imaging is necessary for the diagnosis of NCS.