Successful management of concomitant omphalocele, accessory hepatic lobe, and biliary atresia in a 44-day-old boy.

We report the second case in literature of a boy with the association of large omphalocele, accessory hepatic lobe, and biliary atresia, and the first successful treatment. The patient was submitted to a surgical treatment at 44 days of life, including Kasai procedure, correction of the remnant abdominal wall defect, and removal of a hepatic accessory lobe. The boy evolved with normalization of hepatic function tests. After 15 years of follow-up, the patient is completely healthy, weights 45 kg, and is 1.64 m tall.