Severe pulmonary hypertension is a contraindication for liver transplantation owing to high mortality. However, decision-making regarding the treatment approach for patients with bilateral peripheral pulmonary artery stenosis, typically complicated by elevated main pulmonary artery and right ventricle pressures, can be challenging. Here, we report successful living donor liver transplantation after bilateral pulmonary artery stent implantation in a patient with Alagille syndrome, severe bilateral peripheral pulmonary artery stenosis, and extremely high main pulmonary artery and right ventricle pressures.