Abstract: Objective　To explore the clinical manifestation, diagnosis and treatment of Budd-Chiari syndrome in neonates. Method　The clinical data of Budd-Chiari syndrome in a neonate were retrospectively reviewed and relevant literature was reviewed. Results　The 21-day-old girl was born through vaginal delivery with gestational age of 39 weeks and birth weight of 3150 g. Her clinical manifestations included abdominal distention, hepatosplenomegaly, ascites, repeated hypoproteinemia and low platelet count, similar to sepsis. Imaging examination indicated hepatic segment stenosis of the inferior vena cava (the stenosis segment was about 24 mm in length and 1.59 mm in diameter at the narrowest place). The girl was diagnosed with BuddChiari syndrome and died after abandoning treatment. Conclusion　Neonatal Budd-Chiari syndrome is rare, easily misdiagnosed and has a high mortality.

Key words: 　Budd-Chiari syndrome;　neonate;　diagnosis;　treatment