Biliary atresia (BA) may be characterized as an obliterative cholangiopathy presenting in the newborn period with conjugated jaundice, pale stools, and dark urine. It is usually thought of as an isolated anomaly in otherwise normal infants. However, in a minority, other anomalies may be present, some as defined syndromes, others as a non-random association. The most fully characterized is that of the biliary atresia splenic malformation syndrome seen in about 10% of European and North American series with a typical array of unusual extrahepatic anomalies (e.g., situs inversus, polysplenia, absence of the inferior vena cava, and a preduodenal portal vein). Its underlying genetic background is obscure in most cases. There are other syndromes with a definite link to BA, such as Cat-Eye syndrome and Kabuki syndrome, and still others that may have a link, such as Zimmerman-Laband syndrome.