Abstract
Congenital portosystemic shunt (CPS) is a developmental anomaly of the portal vein system. The disease can cause blood from the portal vein to flow into the vena cava, resulting in various atypical clinical manifestations. Pelvic congestion syndrome (PCS) caused by CPS is particularly rare. A young woman with PCS had an abnormal communicating branch of the left ovarian vein (OV). Her left OV drained normally into the left renal vein, and at the same time communicated with the portal vein, forming an extrahepatic portosystemic shunt. With embolization of her left OV, the patient was cured of PCS.