Synchronous liver and kidney transplantation (SLK) is considered a treatment of choice for an end-stage liver disease patient with irreversible kidney disease. Several perioperative renal supportive treatments, especially continuous renal replacement therapy (CRRT), have contributed to the effective control of hypervolemia and electrolytes and acid-base disturbances leading to high success rate in adults. However, anesthesia for SLK in children is frequently difficult since the CRRT is seldom available because of difficulty in securing large venous lines. In addition, conventional techniques such as venovenous bypass and side-clamping of the inferior vena cava are less applicable in small children causing difficult volume control. Herein, we report a case of SLK in a child with glycogen storage disease type I with a review of literature.