Background: Biliary atresia (BA), characterized by progressive inflammatory destruction of the extra-hepatic biliary tree is one differential diagnosis for jaundice in the newborn. It is a diagnosis which should not be missed, as early surgical intervention is associated with better outcome. In upto 37% cases, this condition can be associated with other congenital anomalies. Case Report: A 2 month old baby girl diagnosed with biliary atresia was found to have a rare collection of coexisting anomalies such as dextrocardia, azygos continuation of the inferior vena cava, multiple spleens, central liver, right sided stomach and pre-duodenal portal vein. The baby underwent porto-enterostomy but she progressed to chronic liver failure. Conclusion: The associated anomalies with BA should be actively looked for in a child diagnosed with this condition, as they will affect the prognosis and the treatment approach.