Congenital anatomic anomalies often present technical obstacles during live
r transplantation. Biliary atresia (BA) is the most common indication for l
iver transplantation in children, and up to 28% of children with situs inve
rsus are complicated by BA. A boy aged 2 years 11 months with BA, situs inv
ersus, and dextrocardia received a liver transplant from his father. The do
nor also had situs inversus; and dextrocardia without other anomalies. Graf
t 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.