Acute fatty liver of pregnancy (AFLP) is a rare liver disease unique to pregnancy potentially fatal for both mother and child. Only a few cases of recurrence have been published. We report a new case. A 27-year-old primiparous patient presented a first episode of AFLP in 1991 at 37 week's gestation. Diagnosis was suspected because of vomiting, thrombocytopenia, and liver function tests abnormalities. It was confirmed by liver ultrasonography and abdominal computed tomography. Clinical and biological improvement was observed after caesarean delivery. Six years later, the woman began a second pregnancy. Liver function tests and complete blood count were regularly checked. At 30 weeks' gestation, recurrent AFLP occurred and caesarean section was performed. Again, diagnosis was confirmed by both ultrasonography and abdominal computed tomography. In 2006, the mother and the two girls, 15 and 8-year-old respectively, were in good health. The study of the HADHA gene, coding alpha subunit long chain 3-hydroxyacyl coenzyme A dehydrogenase (LCHAD) in the patient failed to find mutations, particularly the common mutation c.1528G>C (Glu474-Gln, p.E474Q). In conclusion, after an episode of AFLP, women should be clearly warned of the risk of recurrence and regularly monitored during the next pregnancy, even if the search of HADHA gene mutation is negative.