Résumé : Introduction: Agenesis of corpus callosum (ACC) is commonly diagnosed prenatally. When isolated, it appears to carry a good prognosis but studies are often retrospective and follow-up short. We report a prospective study of 17 children (11 boys, 6 girls) with prenatally diagnosed isolated ACC. Methods: Neuropsychological evaluation was performed each year and results at the ages of 2, 4, and 6 years were compared. Results: Febrile seizures occurred in 3 patients. Median intellectual quotient (IQ) was within the normal range (80-109) and nonrelated to partial or complete ACC, sex, or febrile seizures. Lower median IQ was significantly related to low cultural status. With age, the number of children with IQ in the lower range (80-89) increased and slowness, attentional troubles, and instability appeared. Conclusion: This study demonstrates that if outcome of isolated ACC is favorable, a long follow-up is necessary: with age, IQ in the lower range and behavioral troubles are linked to difficulties in school.