Parenteral nutrition-associated liver disease (PNALD) is frequently observed in patients who require long-term parenteral nutrition. PNALD is diagnosed by clinical presentation, biochemical liver function test, long-term usage of parenteral nutrition, and negative workup for other liver diseases. Pathogenesis of PNALD is multifactorial and includes prematurity, nutritional excess, sepsis, and lack of enteral nutrition. Since PNALD was first reported more than 30 years ago, there have been various attempts to find effective treatments for PNALD. Cyclic parenteral nutrition and use of ω-3 polyunsaturated long-chain fatty acids (ω-3 PUFA) instead of ω-6 PUFA were reported worldwide as effective treatments. This article reviews the literature relating to PNALD.