A case of extrahepatic biliary atresia (EBA) associated with trisomy 18 is
presented. A 1-month-old boy was suspected to have Alagille syndrome with o
bstructive jaundice, a systolic heart murmur, growth retardation, and a sma
ll, pointed chin. However, surgery and chromosomal analysis revealed EBA as
sociated with trisomy 18. Chromosomal examination must be performed in pati
ents with jaundice and congenital anomalies. It is possible that EBA in tri
somy 18 syndrome is due to a chromosomal disorder.