Background: Hepatocellular carcinoma is an uncommon complication described in patients with Budd-Chiari syndrome.
Case characteristics: A 12-year-old boy with Budd-Chiari syndrome, who was earlier treated with Transjugular intrahepatic porto-systemic shunt (TIPS), presented with acute onset hemoperitoneum and hypotension.
Outcome: It was diagnosed to be a case of ruptured hepatocellular carcinoma.
Message: Successful TIPS may not prevent the development of hepatocellular carcinoma, and children with Budd Chiari syndrome should be monitored for the same.