ABSTRACT
Biliary Atresia [BA] is a well-known entity and can present with multiple congenital anomalies. BA is one of the most common conditions in which pediatric liver transplant is performed. Identification of Biliary atresia with situs inversus [SI] has not been documented in Pakistan. We report two such cases. First was an eighty-day-old baby boy, icteric from day of birth. On further evaluation had dextrocardia, SI, gross hydronephrosis [HN] of left kidney and stasis at pelvi ureteric junction [PUJ]. Liver biopsy showed biliary cirrhosis secondary to extra hepatic biliary atresia [EHBA]. The second baby presented at two months of age. Ultrasound abdomen and hepatobiliary scintigraphy confirmed liver in left hypochondrium [SI] and findings suggestive of BA. Echocardiography confirmed SI with mesocardia. In this paper we have described the association of BA with SI in two patients presenting at the pediatric Gastroenterology, hepatology and nutrition unit