Parenteral nutrition-associated liver disease is one of the most dangerous complications associated with long-term use of parenteral nutrition. This complication is especially prevalent in children. There are many theories addressing the etiology of this disease; however, the appropriate treatment and management plan remain elusive. The use of a fish oil-based intravenous lipid emulsion in the management of parenteral nutrition-associated liver disease has been researched recently. Although it has yet to be approved by the Food and Drug Administration, new research shows promising results. This article examines the recent research on management of parenteral nutrition-associated liver disease in pediatrics with fish oil lipid emulsion.