Abstract We present the unique case of a 15‐month‐old male born with biliary atresia and situs inversus totalis and disrupted inferior vena cava who underwent a successful liver transplantation. The patient had previously undergone a failed Kasai procedure and presented with persistent hyperbilirubinemia. The patient was transplanted with a left lateral segment donor having standard arterial anatomy. Technical considerations included identifying completely replaced arterial anatomy in the recipient from the superior mesenteric artery and creating a branch patch between the gastroduodenal artery and HA, anastomosing the donor left hepatic vein to confluences of the donor left, middle, and right hepatic veins, using a “lazy‐S” configuration of portal vein anastomosis, and suspending the allograft to the abdominal wall. Post‐operatively, his liver function tests and total bilirubin normalized and he progressed to tolerating an oral diet with tube‐feed supplementation.