Congenital anatomic anomalies often present technical obstacles during liver transplantation. Biliary atresia (BA) is the most common indication for liver transplantation in children, and up to 28% of children with situs inversus are complicated by BA. A boy aged 2 years 11 months with BA, situs inversus, and dextrocardia received a liver transplant from his father. The donor also had situs inversus and dextrocardia without other anomalies. Graft function was excellent postoperatively, and no significant complications were encountered. This is only the second report of the successful use of a living related donor graft for a patient with BA and situs inversus. This case was particularly rare because the donor also had situs inversus, which made the present procedure more feasible.