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Objective: To assess the validity of our observations regarding Budd-Chiari syndrome (BCS) reported 10 years ago by expansion of our series and close follow-up for an additional decade. Background: 10 years ago we reported our results with what remains the largest clinical experience in Western countries with surgical portal decompression for BCS. Since then our series has expanded to 77 patients and there has been a worldwide explosion of interest in and publications about BCS. Methods: 77 BCS patients were allocated to 3 groups: I - 39 had hepatic vein occlusion alone, treated by side-to-side portacaval shunt (SSPCS); II - 26 had inferior vena cava occlusion treated by mesoatrial shunt in 8 and combined SSPCS/cavoatrial shunt (CAS) in 18; III - 12 had decompensated cirrhosis too late for portal decompression, listed for liver transplantation (LT). An extensive diagnostic workup included angiography with pressures and liver biopsy. Follow-up was 100%, lasting 5-38 years. Results: Group I, long-term survival is 95% with 36 free of ascites, leading lives of good quality 5 to 38 years post-SSPCS. Group II, mesoatrial shunt was discontinued because of a high failure rate, but combined SSPCS-CAS has 100% survival for 5-25 years. Group III, 6 patients (50%) are alive and well for over 5 years post-LT. Serial liver biopsies following portal decompression show long-term reversal of BCS lesions. Conclusions: Long-term survival following surgical portal decompression of BCS has not been equaled by other forms of therapy, medical or surgical. It is imperative to perform surgical portal decompression early in the course of BCS to avoid irreversible liver damage.