目的　探讨新生儿布加综合征的临床表现、诊断及治疗。方法　回顾1例布加综合征新生儿的临床资料，并 复习相关文献。结果　患儿，女，21天。胎龄39周，顺产，出生体质量3 150 g。临床表现为腹胀、肝脾肿大、腹水、反复低 蛋白血症、血小板计数低下，类似败血症表现。影像学检查提示下腔静脉狭窄段长约24 mm，最狭窄处仅1.59 mm，诊断 为布加综合征。放弃治疗后死亡。结论　新生儿布加综合征较为罕见，易误诊，病死率高。

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.