Abstract
Acute fatty liver of pregnancy (AFLP) is a rare but life-threatening condition that develops in the third trimester of pregnancy. AFLP shares similar clinical features with other more common pregnancy-associated conditions. However, early correct diagnosis is important for maternal and fetal survival. Once the diagnosis has been established, immediate delivery and maternal intensive support should be undertaken. Both parents and the infant should be tested for deficiencies of the mitochondrial fatty acid oxidation, especially for long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) deficiency, as many cases of AFLP are related to disruption of this physiological enzymatic pathway.