Acute fatty liver of pregnancy is a rare, but potentially fatal obstetric disorder characterized principally by varying degrees of hepatic failure with an onset typically in late pregnancy. This review outlines the etiopathogenesis and describes the multiorgan involvement that often results in a number of clinical and laboratory aberrations. These laboratory derangements provide distinct features to differentiate from other obstetric complications, such as hemolysis, elevated liver enzymes, and low platelet count (HELLP) syndrome. Once recognized, central to the management of acute fatty liver of pregnancy is delivery planning and meticulous supportive care. One particularly dangerous complication is profound coagulopathy. After delivery, the coagulation defect resolves over 1–2 days, and hepatic and renal function are restored soon thereafter. This report offers anticipated recovery and management strategies for commonly associated complications. Application of these factors has served to decrease mortality from as high as 80% down to 10%; however, given the seriousness of this condition, severe maternal morbidities are frequently associated with this obstetric emergency.