INTRODUCTION: Nutcracker syndrome (NCS) is a rare condition characterized by the entrapment of the left renal vein (LRV) between the superior mesenteric artery (SMA) and the aorta. It was first described in 1950 and the term nutcracker was attributed by de Schepper in 1972. CLINICAL CASE: A 20-year-old female patient was admitted to the urology department with a history of hematuria and left flank pain. Basic hematuria investigation was inconclusive. Further investigation revealed a nutcracker syndrome on retrograde phlebography. Surveillance was the option first chosen. Three years later and after several symptomatic episodes, an intravascular stenting procedure was performed. DISCUSSION: NCS is defined by the compression of the LRV between the SMA and the aorta. Clinical manifestations include left flank pain, hematuria and pelvic congestion syndrome (PCS). Diagnosis is based on history, physical examination, basic lab tests and imaging. Sequence of tests can be composed of Doppler ultrasound (DUS), computed tomography scan (CT scan) or magnetic resonance imaging study (MRI) and retrograde phlebography with pressure gradient to confirm the diagnosis. Management options include surveillance, intravascular and extravascular stenting procedures as well as open procedures. CONCLUSION: NCS is a rare condition that represents a challenge for urologists in terms of accurate diagnosis and proper management.