A case of acute Budd-Chiari syndrome after surgical treatment of polycystic liver disease is described. This complication, presently unreported in the literature, was secondary to the operative interruption of two hepatic veins, while the third was already obstructed by pericystic fibrosis. Preoperative radiologic vascular mapping should be performed in case of voluminous polycystic liver disease when associated liver resection appears likely.