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Ruptured Hepatocellular Carcinoma in a Child with Budd-Chiari Syndrome. 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.