Background: Acute fatty liver of pregnancy is a rare, potentially fatal disease that occurs in the late third trimester or early postpartum period. The case of a woman with acute fatty liver in the second trimester is presented. Case: A 35-year-old woman, gravida 5, para 4, presented at 22 weeks' gestation with nausea, vomiting, malaise, weight loss, and moderately elevated liver transaminase levels. The differential diagnoses included viral gastroenteritis and cholelithiasis. During the next 12 days, her transaminase levels increased, jaundice developed, her sensorium changed, and coagulopathy appeared. After the patient was transferred to a tertiary care hospital, acute fatty liver of pregnancy was diagnosed. The woman rapidly improved after delivery. Conclusion: Although acute fatty liver of pregnancy usually occurs in the late third trimester or early postpartum period, it can occur in the second trimester. (Obstet Gynecol 1999;93:811-3. (C) 1999 by The American College of Obstetricians and Gynecologists.).