Sedation outside operating room for children has increasing importance. Pediatric patients with end stage liver disease are of great importance for various and frequent procedures including gastrointestinal endoscopy, magnetic resonance imaging, computerized tomography, brachytherapy, catheterisation, interventional radiology. Anesthesia plan plays critical role for the success of these procedures. Patient safety, ventilation, hemodynamic responses, side effects of anesthetics on liver and periprocedural analgesia are the main topics of attention. Airway management tools may be helpful because of edema and ascites pushing diaphragm upwards. Nasal capnography enables monitoring spontaneous ventilation. Enlargement of extravascular extracellular fluid and dysproteinemia, effects drug behaviors. Drug elimination half-time as well as context sensitive half-time have to be taken into account and designed individually. The pressure above vena cava inferior, results with decrease in preload, thus reduction in cardiac output. Pulse wave variation monitoring helps for estimating circulating fluid status. Tendency for bleeding can be anticipated with fresh frozen plasma. Patient-controlled analgesia may be a choice of favor but close monitoring required for repeated analgesics. Children's Hospital of Eastern Ontario Pain Scale would be a good monitoring tool for pain.