Background: Encephalocele is defined as the externalization of brain tissue and/or meninges out of the skull through a congenital bone defect. It's a rare congenital malformation with a high risk of mortality and morbidity. Unfortunately, Prenatal diagnostic is still rare. Aims: The aim is to describe the main characteristic of this malformation and the optimal management to improve the prognosis. Case Presentation: we report the case of 32-year-old women diagnosed at 34 weeks gestation with a live fetus with occipital encephalocele. Conclusion: Through this case and the review of the literature we will highlight the diagnostic sources in order to improve the diagnosis and the management.