Abstract
Biliary atresia (BA) is a rare disease in neonates of unknown etiology. BA is defined by the extent of extra- and intrahepatic bile duct destruction, which results in liver deterioration and cirrhosis within the first years of life. Liver transplantation (LT) is the only curative treatment for BA, accompanied by LT-associated risks and complications; however, more than 60 years after it's first report, the Kasai hepatoportoenterostomy (KPE) is still an essential procedure in the sequential management of BA, as the primary surgical treatment option that can achieve long-term survival with a native liver. We highlight the key surgical steps of KPE and discuss relevant aspects.