Liver transplantation for biliary atresia splenic malformation syndrome associated with situs inversus totalis is a challenging task due to the complexity of associated malformations and the technical proficiency required to overcome them. We present the case of a 6-month-old infant who underwent liver transplantation for biliary atresia. A reduced left lateral segment liver graft from a live donor (his mother) was implanted. The postoperative period was uneventful, and the child remained well on follow-up. Thus, such rare congenital anomalies no longer prove to be a deterrent for successful liver transplantation.
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