Extrahepatic biliary atresia (EHBA) is a rare but severe neonatal cholangiopathy characterized by progressive fibroinflammatory obliteration of the extrahepatic bile ducts, leading to cholestasis, biliary cirrhosis, and liver failure if untreated. EHBA typically presents within the first few weeks of life with jaundice, pale stools, and hepatomegaly. The etiology remains multifactorial, involving genetic susceptibility, perinatal viral infections, and immune-mediated injury to the bile duct epithelium. Diagnosis is based on clinical presentation, laboratory findings, imaging, and confirmatory intraoperative cholangiography. The primary treatment is the Kasai portoenterostomy, which aims to restore bile flow by connecting the intrahepatic bile ducts to the intestine. While early surgical intervention can delay disease progression, many patients ultimately require liver transplantation due to ongoing fibrosis and cirrhosis. EHBA remains the leading indication for pediatric liver transplantation worldwide.