Incredible advances in the care of patients born with single-ventricle heart disease have led to significantly better quality of life and survival. Complex surgical palliation culminating in the Fontan procedure is effective and can allow patients to grow and develop similar to their peers. Unfortunately, changes related to Fontan anatomy lead to stress and increased pressure on the liver, known as Fontan-associated liver disease. These stresses can cause scar tissue to form and—rarely—liver cancer to form. Dedicated liver care is vital to providing Fontan patients with the healthiest life possible.