Summary

Recurrent episodes of acute, severe pancreatitis are a feature of the rare WAGR congenital anomaly syndrome. Although pancreatitis is well recognised as a life-threatening association of WAGR syndrome, treatment to prevent pancreatitis is not recommended. We report the successful prevention of hypertriglyceridaemia-associated relapsing pancreatitis in a child with WAGR syndrome with Bezafibrate. In addition to dietary fat restriction, Bezafibrate may be a useful therapeutic adjunct in WAGR related relapsing pancreatitis.