Alagille syndrome is an autosomal dominant syndrome marked by a paucity of interlobular bile ducts with cholestasis, and whose features can include butterfly vertebrae, dysmorphic facies, cardiac and ocular anomalies. Most patients are symptomatic during childhood. As adult patients may require elective liver transplant for cirrhosis and intractable pruritus, liver failure is uncommon in these patients. We present a case of liver failure in an adult with Alagille syndrome. A 32-year-old man with known Alagille syndrome was admitted to the hospital with progressive jaundice and RUQ pain. At that time his MELD was 24 and he was referred for liver transplant evaluation. A month later, he was admitted for hematemesis and hepatic encephalopathy. He had a complicated hospital course with worsening encephalopathy, hepatorenal syndrome (requiring dialysis), and septic shock due to Streptococcus viridans requiring pressors. His peak MELD score during this hospitalization was 42(total bilirubin 47.5, Cr 5, INR 2.0). A CDC high risk liver became available and he underwent an orthotopic liver transplant. This was complicated by bacterial peritonitis from an infected hematoma. However, he recovered well after treatment of his peritonitis, and LFTs were normal one month post-transplant. Allagile syndrome occurs in 1 in 70,000 live births from a JAG1 or NOTCH2 mutation. They have paucity or stenotic bile ducts with progressive cholestasis in 95%, and cirrhosis in more than 20% of cases. Initial treatment includes ursodeoxycholic acid, cholestyramine, fat soluble vitamins and nutrition support. However, 21-31% patients will require liver transplant for severe xanthomatosis, intractable pruritus, and portal hypertension. Most liver transplants are done in the pediatric group. Only 9.7% patients had their first liver transplant beyond 18 years old, and are done electively for cirrhosis and intractable pruritus. Only a few cases were reported due to acute liver failure requiring an urgent transplant, similar to our patient. Alagille syndrome patients may require liver transplantation, usually during childhood. In some cases, transplant may be required in adulthood due to liver cirrhosis and intractable pruritis, and is mostly elective. Liver failure is uncommon in these patients.