Abstract Biliary atresia is an obstructive neonatal cholangiopathy of unknown etiology that produces damage to the parenchyma of the liver and to the intrahepatic and extrahepatic bile ducts. It is the most common cause of neonatal cholestasis and liver transplantation in the pediatric population. In most cases it manifests as an isolated malformation. This article presents the case of a 75 day old patient with biliary atresia associated with abdominal heterotaxy. Biliary atresia syndrome together with splenic malformation has been previously described, as have biliary atresia associated with anatomical malformations of the spleen, pancreas, and heart. It occurs with genitourinary malformations less frequently. The prognosis of patients with biliary atresia KDVVLJQL?FDQWO\LPSURYHGZLWKHDUO\UHFRJQLWLRQRIVLJQVDQGV\PSWRPVDQGWLPHO\SHUIRUPDQFHRIKHSDWRSRU -toenterostomy (Kasai portoenterostomy). Keywords Biliary atresia, visceral heterotaxy Case report INTRODUCTION Biliary atresia is an obstructive neonatal cholangiopathy caused by an inflammatory process that causes progressive fibrosis and obliteration of the intrahepatic and extrahe-patic bile ducts. It is associated with damage to the paren-chyma of the liver (1). It is the most common cause of neonatal cholestasis and biliary obstruction and occurs in 1 in 15,000 to 20,000 live births. If left untreated, the resul-ting cholestasis leads to cirrhosis and liver failure (2, 3). Although in most cases, biliary atresia occurs alone, 10% to 20% of patients have associated vascular and visceral mal-formations (3, 4).Early performance of Kasai Portoenterostomy has a significant impact on the survival of patients with biliary atresia, so primary health care personnel need to be able to use practical tools such as clinical histories so that a multi-disciplinary team specializing in hepatology can make early diagnosis and evaluation (5).This article reports the case of a pediatric patient with biliary cirrhosis and portal hypertension secondary to syndromic biliary atresia, explains the indications for liver transplantation in these patients and emphasizes the value of semiotics for early diagnosis and treatment in cases of neonatal cholestasis which can enable timely interventions that impact positively on the prognoses of these patients.