The aim of this paper was to perform a systematic review with meta-analysis of available scientific evidence regarding the role of different intravenous lipid emulsions (ILE) in the pathogenesis of cholestasis and parenteral nutrition associated liver disease (PNALD).A systematic review of the literature (up to March 2015) identified 23 randomized controlled trials (RCT). Of these, 17 were performed in preterm infants or critically ill neonates with a short duration of intervention, 2 in older children with short term use (following surgery or bone marrow transplantation), 1 in neonates with long term use and 3 in infants and children receiving long term parenteral nutrition (PN).Meta-analysis showed no differences in the rate of cholestasis or bilirubin levels associated with short term use of different ILEs. Due to high heterogeneity of the long-term studies no meta-analysis could be performed. Available studies found that the use of multicomponent fish oil (FO) containing ILE compared to pure soya bean oil (SO) ILE reduced liver enzymes and bilirubin levels in non-cholestatic children on long-term PN and one other RCT found that FO based ILE reversed cholestasis in a proportion of patients. The ESPGHAN Committee on Nutrition concludes that there is no evidence of a difference in rates of cholestasis or bilirubin levels between different ILE for short term use in neonates. The use of multicomponent FO containing ILE may contribute to a decrease in total bilirubin levels in children with IF on prolonged PN. However, well designed RCTs are lacking and long term effects have not been determined.