Alagille's syndrome (AGS) is a multisystem disorder affecting liver, heart, face and skeletal system. Approximately 25% of AGS patients may need liver transplantation (LT) in childhood. Unfortunately AGS patients have multisystem pathologies and they should be carefully evaluated before LT. Especially congenital heart defects in AGS patients may cause hemodynamic effects perioperatively in transplantation surgery. In this case report we aimed to discuss successful anesthetic management for living related donor liver transplantation (LRDLT) in a patient with Alagille's syndrome and severe pulmonary stenosis. Successful anesthetic management of a growth retarded 11-months old, 5110 grams infant for LT is a challenge especially with coexisting cardiac pathologies. Complete preoperative evaluation and careful perioperative monitoring of the patient resulted in stable circulation.