Abstract
We report the case of a late preterm female neonate, born at 36 weeks' gestation, with heterotaxy syndrome, severe congenital heart defects (dextrocardia, situs inversus, left atrial isomerism, complete atrioventricular septal defect (AVSD), and double outlet right ventricle), and biliary atresia. The association of biliary atresia with heterotaxy syndrome is exceptionally rare and adds complexity to the patient´s management. The patient underwent a laparoscopic Kasai procedure, which resulted in significant clinical improvement. This case highlights the importance of early diagnosis, a multidisciplinary approach, and timely surgical intervention in managing rare and complex conditions.